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Hearing Aids Inside Out

Hearing Aids Inside Out

Have you recently experienced hearing loss? Most probably you need hearing aids, but don't know much about them. To learn everything you need to know about hearing aids, read the eBook, Hearing Aids Inside Out. The book comprises 113 pages of excellent content utterly free of technical jargon, written in simple language, and in a flowing style that can easily be read and understood by all.

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Breaking The Sound Barriers

Living with a deaf child is one of the hardest things that you will ever deal with in your life; the barriers to their learning and potential happiness can seem like you will never cross them. However, it is easier than you thing to learn how to communicate with your deaf child on a level that you both are able to understand. This ebook guide is designed to teach you how to truly communicate with your child so that it minimizes frustration for both of you, and allows you child to learn at a much higher level than almost anyone would have believed. You will also hear the success stories of deaf children that have gone on to achieve amazing success in their lives. Having a deaf child is a blessing, not a curse. This guide will show you how best to love and care for your child!

Breaking The Sound Barriers Overview

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Possible Causes of Treatment Related Hearing Loss

Mechanical damage to the axons or ischemia of the cochlea or auditory nerve may lead to immediate postoperative hearing loss during microsurgery. Stretch injury to the transitional Obersteiner-Redlich zone of the cochlear nerve has been suggested as the cause of hearing loss during tumor removal.32 We did not observe immediate hearing loss in any patient after radiosurgery. If hearing impairment is noted, usually it is gradual. Early hearing loss after radiosurgery (within 3 months) is rare and may result from neural edema or demyelination. Delayed hearing loss after microsurgery could result from the vascular or fibrotic changes or the development of endolymphatic hydrops after damage to the endolymphatic duct during surgery. The precise mechanism of delayed hearing loss after radiosurgery is still unclear. Perhaps delayed obliteration of microvessels or even direct radiation injury to the axons, in patients treated with higher radiosurgical doses, could account for hearing loss....

Cholesteatoma Hearing Loss

Scan Hearing Loss

Figure 3.38 Polyp in the external canal in a child presenting with continuous otorrhea and hearing loss. A CT scan (Fig. 3.39) shows the presence of a soft-tissue mass eroding the intercellular septae of the mastoid and the ossicular chain, suggestive of cholesteatoma. This was confirmed during surgery. Figure 3.38 Polyp in the external canal in a child presenting with continuous otorrhea and hearing loss. A CT scan (Fig. 3.39) shows the presence of a soft-tissue mass eroding the intercellular septae of the mastoid and the ossicular chain, suggestive of cholesteatoma. This was confirmed during surgery. Basal cell carcinoma is more frequent in the auricle, particularly in subjects with long exposure to the sun. On the other hand, squamous cell carcinoma accounts for about three quarters of invasive tumors of the external auditory canal and the middle ear. In about 11 of cases, cervical lymph node metastases are present at the time of diagnosis. The most common symptoms include...

Issue 1 Impact of Unilateral Hearing Loss

In the past, clinicians expressed little concern about unilateral loss of hearing. Recently, research has shown that children with a significant unilateral sensorineural hearing loss and presumably a significant unilateral conductive hearing loss do experience a variety of auditory, linguistic, and cognitive difficulties that may impact educational progress.1 Impaired sound localization1-3 and speech recognition in background noise do occur with unilateral hearing loss and may underlie these difficulties. Interestingly, the speech recognition problem occurs regardless of whether the speech stimuli are directed toward the normal or hearing-impaired ear.4'5 These findings have obvious implications for the student in the typical classroom environment.

Demographic Influences On Ci Performance In Children With Prelingual Deafness

Age at implantation, length of cochlear implant use, communication mode, and amount of residual hearing before implantation are all demographic factors that have been shown to influence performance results in children with congenital or prelingual deafness. However, age at onset of deafness does not influence performance for this group of children. The speech perception performance of pediatric CI recipients with congenital deafness is similar to that of their peers with adventitious deafness acquired before age 3 years.43 These results suggest that both groups of children can benefit from similar intervention strategies.

NOC Risk Control Hearing Impairment

Assess history of chronic otitis media, brain infection, use of ototoxic drugs, rubella or other intrauterine infections (viral), congenital defects of ear or nose, presence of deafness in family members, hypoxemia and increased bilirubin levels in low-birth weight infants. possible risks for conductive or sensorineural hearing loss. Alert parents to behavioral cues indicating hearing impairment. acuity and or loss and type of hearing loss. Promotes identification of hearing loss for correction before development is affected. Encourage parents to notify school nurse and teacher of degree of hearing loss and methods of communications used by child.

Hearing Loss

Bone Anchored Hearing Aid

Most hearing loss is easy to diagnose as either a well-defined conductive or sensorineural type. (Mixed hearing loss may occur, but this diagnosis is usually non-contributory, and the term is better avoided.) Lesions to the left of the red line (Fig. 1.16) cause conductive hearing loss, and are frequently curable. Hearing loss to the right of the blue line is due to a sensorineural lesion, and is usually not so amenable to treatment. Fig. 1.16 Conductive and sensorineural hearing loss. Hearing loss is either conductive or sensorineural in type. It is an essential basic step in diagnosis of hearing loss to distinguish between these two. Sensorineural hearing loss is either due to a cochlear or retrocochlear lesion. Fig. 1.16 Conductive and sensorineural hearing loss. Hearing loss is either conductive or sensorineural in type. It is an essential basic step in diagnosis of hearing loss to distinguish between these two. Sensorineural hearing loss is either due to a cochlear or...

Hearing losses

Approximately 10 of any nation's population has a hearing loss of one type or another. It is estimated that 21.9 million U.S. adults have hearing loss, 15.2 million of those having moderate to severe loss. About 4.7 million use hearing aids and 2.3 million are unsatisfied with their hearing aids. There are two basic mechanisms for hearing loss. In one, conductive hearing loss, the mechanically conductive pathway to the inner ear is impaired, although the inner ear works fine. The second loss mechanism, sensorineural hearing loss, involves the nervous system, meaning that the conduction to the inner ear is operative but the transduction mechanism to a nerve action potential by hair cells is impaired the sound signal does not get conveyed to the auditory cortex of the brain. When devices are implanted to amplify or restore hearing, they deal with mechanics or neural stimulation, depending upon the hearing loss type. In some individuals, there is a mixed hearing loss, meaning both...

Otitis Media with Effusion Socalled Glue ear in Children

A middle-ear effusion is a common cause of conductive hearing loss. It may occur when either a head cold or barotrauma interferes with eustachian tube function, and it often follows acute otitis media. A postnasal space neoplasm may also cause eustachian tube dysfunction, and is to be excluded in any adult with a persistent otitis media with effusion.

Management of Cholesteatoma

Most otologic surgeons prefer to avoid operating on an only hearing ear. Preoperative scanning can not only help determine the risk of observation, but if no evidence of disease is seen in the middle ear space, a modified radical mastoidectomy (without tympanoplasty) can be justified. Some surgeons believe that avoiding surgery in the middle ear space reduces the risk of postoperative hearing loss.

Surgical Repair of the Traumatized Facial Nerve

Radiographic findings are extremely important in the guidance of the surgical exploration in repair of a traumatized facial nerve. Most injuries are in the vicinity of the geniculate ganglion, specifically, the proximal tympanic segment, the geniculate ganglion, or the labyrinthine segment.14 Surgical exploration of the facial nerve after temporal bone trauma is initially performed as a transmastoid approach. Canal wall-up mastoidectomy with facial recess is often adequate for surgical exploration and decompression of the facial nerve from the proximal tympanic segment through the distal vertical segment. Exploration ofthe geniculate ganglion or labyrinthine internal auditory canal segment can be performed via a middle fossa craniotomy. If the patient has suffered total sensorineural hearing loss from trauma, a translabyrinthine approach is preferable to the middle fossa approach to obtain total facial nerve exposure without temporal lobe retraction in the acute head injury scenario.

The Tympanic Membrane and Middle

Tympanic Membrane Perforation Healed

The defect is frequently slit-shaped (c). Pain and transient vertigo at the time of injury are followed by a tinnitus and hearing loss. The defect is frequently slit-shaped (c). Pain and transient vertigo at the time of injury are followed by a tinnitus and hearing loss. Fig. 2.58 Central perforation. Acute otitis media with pus under pressure in the middle ear may rupture the drum, and although healing usually occurs, a permanent perforation can result. These perforations are usually central. A small perforation may be symptom-free, but episodes of otorrhea with head colds and after swimming are common, along with a conductive hearing loss. Fig. 2.60 A posterior marginal perforation of the eardrum, taken with the fiberoptic camera, showing the round window and head of the stapes. A thin fibrous connection can be seen (arrow) which connects to the necrotic long process of the incus. This type of ossicular discontinuity is a common cause of conductive hearing loss following otitis...

Transtympanic Gentamicin Ablation

PLF-related symptoms in patients with unilateral PLFs and a normal opposite ear, ablation of inner ear hair cells does not address the mechanical problem of the open fistula or otic capsule defect. The instillation of ototoxic agents into the middle ear of the PLF patient is associated with a greater risk of ototoxic hearing loss and destruction of vestibular hair cells than would occur in a nonfistulous ear. The treatment also leaves the fistula open, placing the patient at risk of potentially serious complications (see below). For these reasons, we believe that treating the symptoms of active PLF with transtympanic ototoxic agents is inappropriate.

ENT Examination

Headlamp Used Indirect Laryngoscopy

Fig. 1.1 The instruments needed for an ENT examination The laryngeal and postnasal mirrors require warming to avoid misting, and hot water or a spirit lamp is necessary. An angled tongue depressor or wooden spatula is needed for examining the oropharynx and postnasal space. Angled forceps are used for dressing the nose or ear. A tuning fork is essential for the diagnosis of conductive or sensorineural (perceptive) hearing loss. A C1 or C2 (256 or 512 cps) is needed. The very large tuning forks used to test vibration sense are unsatisfactory, and may give a false Rinne test. AJobson-Horne probe is widely used in ENT departments. A loop on one end is for removing wax (and foreign bodies) from the ear or nose. Cotton wool attached to the other end is used for cleaning the ear. Fig. 1.1 The instruments needed for an ENT examination The laryngeal and postnasal mirrors require warming to avoid misting, and hot water or a spirit lamp is necessary. An angled tongue depressor or wooden spatula...

Chronic Otitis Media With Effusion

Otitis Media With Effusion Adults

Middle-ear fluid, if persistent, may cause permanent changes in the drum. An otitis media with effusion can cause hearing loss for decades, and the diagnosis is frequently overlooked in a long-standing hearing loss. Impedance audiometry helps in diagnosis. Fig. 2.82 Grommet occluded with exudate. Insertion of a grommet in these chronic adult cases may restore hearing, but frequently either the lumen of the grommet becomes occluded with exudate, which may extrude through the tube into the meatus, or a constant otorrhea occurs. There is no successful treatment at present for chronic otitis media with effusion when this fails to respond to insertion of a grommet. A further problem with chronic otitis media with effusion is the return of middle-ear fluid with hearing loss when the grommet extrudes. A larger flanged grommet (long-term grommet) which remains in position longer, and periodic replacement are the present remedies.

Complications Of Plfs

Curiously, PLF-related complications are rarely, if ever, discussed in the literature. In addition to the comorbid conditions discussed above, there are three significant complications of PLFs Hearing loss, vestibular function loss, and meningitis. Hearing loss is a complication of a PLF and should never be regarded as either a primary consequence or inevitable result of PLF. Hearing loss complicating PLFs is a true otologic emergency. Sensorineural hearing loss indicates hair cell damage and or death, whereas a conductive or mixed loss (often seen in cases of post-traumatic PLF) indicates a superimposed middle ear component. Probability of improvement in SNHL after PLF closure is 50 50 at best,62 and the likelihood of improvement diminishes rapidly with time.

Aural Atresia Longer Term Hearing Status

Many tympanoplasty procedures involving reconstruction of the ossicular chain. Like chronic ear surgery, some decrement in hearing is to be expected over time9'13'14 (Table 70-2) and revision surgery may be necessary. In my experience, approximately 60 of patients will achieve an excellent initial hearing result of 10 to 25 dB SRT. Approximately 75 of these individuals will maintain this level of hearing longer term (follow-up mean 2.8 years, range 1 to 7.5 years). Considering all patients, revision surgery will be necessary in approximately one-third of cases. The most frequent complication requiring secondary surgery is stenosis of the external ear canal. Even for the highly successful stapedectomy, approximately 20 of patients will experience a significant recurrent conductive hearing loss after successful air-bone gap closure if follow up extends beyond 5 years.15

Medical Management Options for Otitis Media Antibiotics

Acute otitis media and have no suspected significant hearing loss are candidates for chemoprophylaxis, typically using low-dose amoxicillin and sulfasoxazole. Numerous studies have looked at this issue with no definite conclusions having been determined.31 Williams et al.31 performed a meta-analysis of prior chemoprophylaxis studies, specifically looking at the efficacy of chemoprophylaxis in otitis media. The study did demonstrate a small short-term benefit for the management of recurrent otitis media. However, there did not appear to be any long-term benefit to this treatment strategy. Paradise16'32 stated that physicians should reexamine the role of chemoprophylaxis and the treatment of recurrent otitis media due to the increased incidence of bacterial resistance. He advocates refraining from administration of chemoprophylaxis altogether, or possibly limiting the role of chemoprophylaxis in otitis-prone children just during upper respiratory tract infections.

Surgical Management Options

The primary surgical therapy for chronic otitis media remains placement of tympanostomy tubes for pressure equalization and drainage of the middle ear space. Numerous publications have demonstrated the efficacy of tympanostomy tubes for the treatment of chronic otitis media with effusion and recurrent otitis media.38,40-43 The Academy of Otolaryngology, in its 1995 Clinical Indicators Compendium,44 listed the following indications for placement of tympanostomy tubes (1) middle ear effusion present for 3 months or more (2) otitis media with effusion associated with hearing loss of > 30 db (3) chronic severe tympanic membrane retraction (4) impending intracranial complications associated with otitis media and (5) recurrent otitis media with more than three episodes within a 6-month period, or more than four episodes within a 12-month period. It should be emphasized that these indications are guidelines and a decision to place tympanostomy tubes should be individualized for each...

Evidence That Antimicrobial Agents Are Indicated for Treatment of Acute Otitis Media

Shorten time with middle ear effusion (hearing loss)9 Middle Ear Effusion Outcome Kaleida and colleagues9 evaluated amoxicillin or placebo for management of nonsevere acute otitis media. (The distinction between severe and nonsevere disease was based on an otalgia scoring system and the child's temperature subjects in the severe category were not randomized to receive only placebo.) At the completion of the 2-week amoxicillin treatment, there were statistically fewer children with middle ear effusion (47 ) when compared to those who received placebo (63 ). Since presence of middle ear effusion is associated with a conductive hearing loss, the administration of an antimicrobial agent reduces the time with hearing loss, which may have implications for child development.

To Treat or Not to Treat with Antibiotics

Many question the need to treat otitis media with effusion, because the effusion will resolve in most children without active treatment in 2 or 3 months. Nevertheless, treatment may be indicated in some children, because there are possible complications and sequelae associated with this condition. Since hearing loss of some degree usually accompanies a middle ear effusion, treatment may be warranted when longstanding impairment in hearing is present. Although the significance of this hearing loss is still uncertain, such a loss may impair cognitive and language function and result in disturbances in psychosocial adjustment. Important factors that should be considered when deciding to treat or not to treat are listed in Table 78-5.

Annelle V Hodges Thomas J Balkany Stacy L Butts and Shelly

In spite of repeated FDA approval and NIH support for use of cochlear implants in children, a strong body of opposition to pediatric use of the device has remained. Few if any other proven treatment options for treatment of major pedi-atric health disorders have received the type and intensity of negative response which has accompanied use of the cochlear implant in children. As suggested by the title of this chapter, opposition has centered on resisting implantation of the con-genitally deafened child. That adults and postlinguistically deafened children can benefit from implants has been acknowledged by most of those who continue to oppose its use in the con-genitally deaf child. Primary opposition has come from members of the deaf world, a subset of the deaf community who hold more extreme views on the nature of deafness. They hold that deafness is not a disability, but is rather a natural human variation and therefore does not need to be treated medically.3 Support for their...

Congenitally Deaf Children Cannot Benefit from Implants

Studies such as those conducted by Shepherd et al.,8 Matsushima et al.,9 Lousteau,10 and Hartshorn et al.11 have provided evidence that suggests otherwise. Animal studies have shown that even in the presence of the auditory deprivation caused by congenital deafness, some cochleotopic organization remains and that electrical stimulation may both prevent degenerative changes in the neural pathways and may in fact produce morphologic and physiologic changes that improve function. Obviously, the longer the period of deprivation, the greater the negative impact the positive benefit of the implant decreases. This has implications when considering implantation of older congenitally deaf children and adolescents who have not been consistent users of amplification.

Ethical Opposition to Cochlear Implants in Congenitally Deaf Children

In order to understand why cochlear implant technology is perceived as a significant threat by the deaf community, it is important to note that 90 of deaf children are born to hearing parents and 97 have at least one hearing parent.3 If the deaf world is to continue, the children of hearing parents must continue to embrace the language and culture of deafness. Traditionally, this culture and language have been absorbed by deaf children at residential schools where teachers, house parents, and peers are generally themselves members of the deaf community. If parents are given a safe and effective means of enabling their children to remain at home and enter the educational mainstream, many children could be lost to the deaf community. Ultimately, this could have a serious impact on the perpetuation of deaf culture and language. The desire of parents to keep their children at home received national support in the passage by Congress of Public Laws 94-142 and 99-457. These laws ensure that...

Richard T Miyamoto Karen Iler Kirk and Laurie S Eisenberg

In 1990, the Food and Drug Administration (FDA) first gave approval for cochlear implantation in children aged 2 to 18 years. Initially, children who received a cochlear implant (CI) had total profound deafness, and most were older than 5 years of age. Early speech perception results demonstrated that congenitally or prelin-gually deafened children with a CI displayed substantial closed-set abilities (e.g., wherein children identify a word by selecting from a limited set of response alternatives), but only minimal open-set spoken word recognition abilities (i.e., in which no response alternatives are provided).1'2 Since then, as cochlear implantation has been extended clinically to younger children, and with continued improvements in electrode design and signal processing,3-6 pedi-atric CI recipients have achieved much higher levels of open-set word recognition.7-13 For example, Eisenberg and colleagues14 reported mean Phonetically Balanced Kindergarten word lists (PB-K) scores of...

Results Of Cochlear Implantation In Children Nucleus Cochlear Implant Systems

One of the first large-scale reports of pediatric performance with the Nucleus cochlear implant was presented by Staller et al.2 These investigators presented speech perception data from 80 children with the Nucleus 22-channel cochlear implant system who were tested as part of the FDA clinical trials. The mean age at onset of deafness was 2 years, 8 months, and the mean age at implantation was 9 years, 10 months for this group of children. The children's performance was classified by the highest category of speech perception achieved. Comparisons were made between their speech perception performance preimplant and again at 12 months postimplant. After 12 months of cochlear implant use, 63 of children showed significant improvements in the closed-set speech perception tasks and 46 of children demonstrated significant improvements on at least one open-set speech perception task. However, open-set speech abilities were still relatively modest. Similar word-recognition results were...

Minimum and Maximum Age for Implantation and Hearing Aid Benefit

There are both national initiatives and regional efforts to establish universal hearing screening in the United States.4'5 The stated goal of these initiatives is to identify all infants with early onset of hearing loss by 3 months of age and to accomplish hearing aid fitting by 6 months of age for those infants identified with significant hearing loss. The point of these initiatives is to take advantage of the child's natural facility for the acquisition of speech and language during the first year(s) of life, a facility that does not depend on exposure to spoken language but that applies across any mode of language expression. If spoken language is the goal, we know that the sooner sensory evidence from the aided auditory channel is incorporated into learning for the child with congenital deafness, the better the outcomes will be. According to this argument, implantation during the first year of life, but certainly before 2 years of age, seems logical. If this approach is taken,...

Family and Educational Support

Establishing communication with, and developing language in, a child with congenital deafness is one of the most challenging problems to confront a family. The vast majority of parents (90 ) who have a deaf child do not know anything about the habilitation process and do not know how to use sign language or how to otherwise communicate with their child. Furthermore, outside of populated areas, strong educational programs or intervention teams, or both, are often not available to children and their families. If these support systems cannot be identified, implantation is not advisable. Use of sign language, per se, should not recommend against implantation. Measurable gains in language performance, including acquisition of intelligible speech, have been noted for children with implants who are in programs or homes in which sign language is used in addition to speaking and listening. Sadly, however, most families do not develop into competent communicators with their child in any mode,...

Deaf Culture and Cochlear Implants

One point in this controversy that must be recognized by any family or professional involved in the implant process, however, is that many people who do not talk (and only use signs) are well educated, literate, and gainfully employed, have a productive family life, raise well-adjusted children whatever their hearing status, and otherwise contribute to society. They would argue that deafness does not have to be considered a condition to be fixed. It should also be recognized that the deaf community is not monolithic. Some parents who are themselves con-genitally deaf, who use sign language and function socially in

Perceptions of the Child

As societal perspectives about people with disabilities have been modified, whether through federal legislation or through changes in society itself, we have begun to separate the disability from the person. Reading about the accomplishments of a woman who is both deaf and blind, or having an adult with Down's syndrome on a popular television show, or seeing a Miss America who happens to be deaf successfully handle the complexities of modern press coverage, point out how alike we are and how extraordinary some of us can be despite intellectual or sensory disabilities. It should also highlight the importance of being comfortable with oneself, with one's strengths and limitations. We are beginning to understand more clearly how our earliest experiences affect our adjustment and self-perception as adolescents or adults. How well do we understand the implications of implant surgery on the child's perception of themselves, as someone who needs to be fixed, rather than accepted as a child...

Implants in Children with Multiple Disabilities andor Auditory Neuropathy

Our ability to implant children with a cochlear device has no more serious potential for damage and financial waste than in instances in which the child is deaf but has additional disabilities. It must be admitted, if not generally acknowledged, that children with multiple disabilities, including mental retardation or severe language-learning difficulties, have been implanted already. In some children who are deaf and blind but otherwise intact, the decision to implant may be a good one. However, the parents of children with multiple disabilities, including deafness, are among the most vulnerable as they seek help for the child whose future may seem bleak. Everyone must understand the implant's role as only one factor in the habilitation and long-term development of any child with deafness. Unless this is clear to all parents, some well-meaning surgeons will continue to be persuaded that an implant is critical for a particular child. Professional implant teams that include...

Outcome Data Controversy

For the past dozen years or so, outcome data for children with cochlear implants have moved from case studies showing considerable promise to both summative and evaluative data for dozens of children with profound hearing loss, including those with congenital deafness. The population of students with implants has grown steadily in schools that emphasize auditory oral education and may constitute as many as one-half of the children currently enrolled in those programs. Part of the impetus for this trend may be the outcome research showing that many children with multichannel cochlear implants outperform children with profound hearing losses who are considered to be very good hearing aid users on a variety of speech perception and speech and language production tasks.7'16'17 This research is generally in a quantitative style and provides answers about changes in discrete test performance both pre-and postimplant. Efforts to examine the question of improvement in quality of life, as well...

Neural and Cortical Plasticity Reorganization

That has never been stimulated, as in children with congenital deafness, remains. Data concerning adults who begin to wear a hearing aid on a long unaided ear suggest some answers, as many seem to recover speech perception abilities after a period of accommodation, but these are persons who lose hearing as adults and thus have well-developed auditory and language abilities (and brain organization) to assist with accommodation. Positron-emission tomography (PET) scan studies in cochlear implant users20 and other research of central auditory effects with electrical stimulation21 seem to offer the possibility that peripheral stimulation does have the effect of (re)activating central auditory pathways.

Management of the Unilateral Atretic

No one would argue that surgery for congenital aural atresia has the same potential for hearing restoration as does stapedectomy few middle ear procedures involving the middle ear ossicles do. But is the success rate so modest that unilateral cases are best observed, at least until the individual is an adult and can make his or her own decision Most otologic surgeons, even experienced ones, would agree that surgery for congenital aural atresia is challenging. Some submit that this procedure is inappropriate for unilateral cases, whereas others express no hesitation in recommending intervention to selected patients. The basis for this controversy has not been clearly articulated in the literature. In many cases, the bias has simply been inherited as part of the philosophy of one's training program. In order to provide more objectivity to this issue, several possible areas of concern need to be explored (1) impact of unilateral hearing loss, (2) probability of achieving binaural hearing...

Conservative Treatment

In the absence of complications, such as sensorineural hearing loss, treatment of active, uncomplicated PLFs should always begin with conservative treatment. About 85 of patients will improve with 6 weeks of bed rest.62 The goal of bed rest is to minimize movement across the defect causing PLF. This immobilization technique is accomplished by minimizing changes in intracranial, intrathoracic, and or intra-abdominal pressure and is analogous to the principles of wound healing employed by all successful surgeons.

Posterior Petrosectomyneurotologist

Ried inferiorly to expose the jugular bulb. Exposing the middle and posterior fossa dura and the intervening superior petrosal sinus reveals the superior semicircular canal (Fig. 82-4B). For patients with irreversible hearing loss a labyrinthectomy may be performed to expand the exposure.

Authors Perspective

(1) addressing patient characteristics, comorbidities, and behaviors (2) considering practical ear characteristics (mesotympanic fluid and mucosa, and eustachian caliber) and (3) antiseptic ear cleansing, and irrigating until accessible mesotympanic fluid is cleared. In infants with cleft palate, assuming that behavioral audiometry has not revealed worse than a mild loss (in the better-hearing ear), we like to defer tympanostomy tube placement until the anesthetic at which the cleft is repaired, typically at 9 to 12 months of age. In acquired immunodeficiency syndrome (AIDS) patients, and in patients with immotile cilia syndrome, we try to avoid placing tympanostomy tubes for persistent non-suppurative fluid with clinically significant bilateral hearing loss, amplification seems appropriate. Bottle feeding in the supine position is discontinued before tympanostomy tube insertion. Graduation from the bottle by the first birthday is encouraged. The otitis-exaggerating effects of day...

Preoperative Imaging

When a patient wishes to avoid operation If an individual is a poor surgical candidate or for other reasons wishes to avoid operation, CT scanning can help assess the risk of conservative management. The presence of extensive disease, labyrinthine fistula, encroachment around the fallopian canal, and involvement of the oval window niche, increase the risk of expectant management, and the patient should be so advised. Most otologic surgeons prefer to avoid operating on an only hearing ear. Preoperative scanning can not only help determine the risk of observation, but if no evidence of disease is seen in the middle ear space, a modified radical mastoidectomy (without tympanoplasty) can be justified. Some surgeons believe that avoiding surgery in the middle ear space reduces the risk of postoperative hearing loss.2 3. For preoperative risk assessment In some circumstances, it may be especially important to patients or parents to have some assessment of the risk of ossicular involvement...

Laser Safety

Laser damage to the inner ear structures or the facial nerve has been a concern since both before and after lasers proved clinically effective in otologic surgery. Silverstein et al.33 reported adverse results in their first two cases using an argon laser fiberoptic hand piece. In a revision stapes procedure, their first patient developed a discrimination loss of 40 . In a second case, a total hearing loss resulted when a large granuloma of the tympanomeatal flap filled the middle ear and grew into the oval window.

Results

The argon laser has been used for stapes surgery for two decades, and the fiberoptic argon laser has been used for more than one decade. The question of visible light laser safety has been answered by the paucity of reported clinical complications in the literature. In a review of more than 2200 cases, there were no cases of significant sensorineural hearing loss or facial nerve injury.34 In the report of our first 43 patients undergoing fiberoptic argon laser stapedotomy, 72 of patients had closure of the air-bone gap to within 5 dB, 19 had closure to within 10 dB, and 9 had closure to within 15 dB.14 In our review of 300 patients, our results had changed very little. Closure of the air-bone gap to within 5 dB was found in 73 , 20 had closure to within 10 dB, and 7 had closure to within 15 dB.21 These results are similar to those reported for the CO2 laser (91 within 10 dB) and the KTP-532 (90 within 10 dB).27'29

Other Surgery

The treatment of hearing loss in Meniere's disease. Otolaryngol Clin North Am 1997 30 1123-1144 One theory of Meniere's disease is that increased endolym-phatic pressure causes the symptoms as well as the histopatho-logic features. According to this theory, pressure buildup leads to membrane ruptures, so that potassium-rich endolymph gains access to the sensory and neural structures, causing sudden or fluctuant hearing loss and episodic vertigo. This theory also suggests that pressure buildup develops as a result of altered resorption of fluid by the endolymphatic sac. Abnormalities that lead to this altered resorption include perisaccular and vestibular epithelial fibrosis, altered glycoprotein metabolism, viral infection of the inner ear, and autoimmune-mediated dysfunction of the sac. Bony narrowing of the endolymphatic duct might also contribute to the obstruction and dysfunction of the sac. In this era of managed care with declining health care revenues,...

Acute Otitis Media

Inner Ear Loss

Earache with conductive hearing loss and fever accompanying a head cold characterize acute otitis media. The drum is red and the landmarks are obscured drum distension and pulsation may be seen. Barotrauma causing marked middle-ear pressure change, e.g., from diving or flying, may also disrupt the stapes footplate ligament or rupture the round window, causing perilymp fluid from the inner ear to pass into the middle ear (arrow) a perilymph fistula. A fluctuating sensorineural hearing loss and vertigo ensue. Otosclerosis Hearing Loss Due to Fixation of the Stapes Bone Otosclerosis Hearing Loss Due to Fixation of the Stapes Bone Fig. 2.89 Otosclerosis. This is a common cause of bilateral symmetrical conductive hearing loss in adults. Otosclerosis is familial and more common in women (otosclerotic hearing loss increases during pregnancy, which may account for the apparently higher incidence in women). Patients frequently notice paracusis, in which there is improved hearing with...

Middle Ear Effusion

Picture Middle Ear Effusion

Glue ear is common between the ages of three and six years, and uncommonly persists after 11 years. The hearing loss is often slight and varies with colds. The self-limiting nature of the condition calls for conservative treatment, but glue ears are not to be ignored. A marked and persistent hearing loss, interfering with schooling, necessitates surgery. Episodes of transient otalgia are common with glue ears, and frequent attacks of acute otitis media may occur. The drum may also become retracted and flaccid with prolonged middle-ear fluid. These features may necessitate insertion of a grommet to reventilate the middle ear. A similarly blue appearance of the tympanic membrane is seen following injury when bleeding occurs in the middle ear (hemotympanum). The conductive hearing loss associated with this injury resolves with resorption of the middle-ear hematoma. A persisting conductive hearing loss following injury, however, suggests injury to the ossicles with an ossicular...

Diagnostic Testing

Battery, the audiogram provides the most consistent information to consider the diagnosis of perilymph fistula. The hearing loss is usually a sloping high-frequency sensorineural hearing loss with impaired speech discrimination. Progressive sensorineural hearing loss with or without deterioration of speech discrimination is a finding that should bring perilymph fistula to the forefront of the differential diagnosis. Electro-cochleography has not been shown to enhance the ability to confirm the diagnosis, as a positive finding of the summation potential action potential ratio of > 0.5 was identified in 52 of patients with confirmed perilymph leaks.8

Otitis Externa

Otitis Externa Bullosa

In protracted cases of this type of otitis externa, the skin of the deep mea-tus and drum becomes thickened and funneled with meatal atresia. The resulting conductive hearing loss is extremely difficult to treat surgically once this condition is quiescent. Examination shows hemorrhagic blebs on the drum and meatus, similar to the vesicular eruption of herpes. If there is pyrexia with a conductive hearing loss, the otitis externa is associated with an otitis media, and systemic antibiotics are necessary. In the absence of pyrexia and hearing loss, this condition settles spontaneously without treatment.

Patient Selection

It is important to demonstrate a patent oval window on CT. An absent oval window is more common in minor malformations21 (i.e., mild microtia, external auditory canal and tympanic membrane normal or only slightly small, conductive hearing loss) but can occur in aural atresia. Drilling a neo-fenestra, especially when the tympanic segment of the facial nerve is displaced inferiorly, and reconstructing with a prosthesis are associated with increased risks and decreased probability of long-term hearing improvement.

Summary

Most otologic surgeons would explore the middle ear of a child with a unilateral conductive hearing loss secondary to trauma, infection, or presumed ossicular fixation (e.g., malleus or stapes). Why, then, the reluctance, or controversy, regarding aural atresia, which, fortunately, is unilateral in most patients Certainly, the hearing loss resulting from aural atresia is significant both in terms of severity (i.e., maximal conductive deficit) and, as discussed above, in its potential impact on auditory and linguistic development. 4. Tharpe AM, Bess FH. Identification and management of children with minimal hearing loss. Int J Pediatr Otorhinol 1991 21 41-50 5. Bess FH, Tharpe AM, Gibler AM. Auditory performance of children with unilateral sensorineural hearing loss. Ear Hearing 1986 7 20-26 7. Ferguson MO, Cook RD, Hall JW III, et al. Chronic conductive hearing loss in adults effects on the auditory brainstem response and masking-level difference. Arch Otolaryngol Head Neck Surg 1998...

Patient Evaluation

We cannot overemphasize the importance of complete audiologic evaluation even in the child with unilateral atresia with or without microtia. The infant with bilateral or unilateral atresia microtia should undergo brain stem auditory response testing of both ears early in infancy to evaluate sensorineural function. Bone conducting hearing aids should be placed on the child with bilateral atresia as early as possible. In addition to inner ear anomalies that are occasionally seen with atresia cases, there is a reported incidence of conductive hearing loss in the apparently normal contralateral ear of up to 27 , with a sensorineural loss in as many as 18 .4

Timing of Surgery

Older patients in whom mild presbycusis in the contralateral ear has begun to develop, or patients who suffer hearing loss in the normal ear, often seek surgical evaluation for correction. They realize how important binaural hearing is to them once the hearing becomes compromised in the normal ear. These patients, with favorable anatomy, are certainly candidates for atresiaplasty. Our oldest atresia patient was 61 years of age.

Decision Making

In the case of bilateral aural atresia, the primary concern is that of the development of normal speech and language. These children should be fitted early on with bone anchored hearing aids, and surgery should be strongly considered. In the case of unilateral aural atresia, a complete audiologic evaluation should be performed. Surgery is only a consideration if there is normal or near-normal sensorineural hearing loss in the atretic ear. Historically it has been felt that if the patient has normal hearing in one ear, then the effect on language and speech will not be greatly affected by the loss of hearing in the other ear. This premise has recently been called into question and there is debate currently over whether unilateral hearing loss can in fact create linguistic and cognitive deficits.6

Salivary Glands

Pictures Empacted Salivary Glands

Acute viral parotitis is a common infection, and the diagnosis is usually obvious. Well-defined tender swelling of the parotid gland, first on one side and shortly after on the other, with associated trismus and malaise, are characteristic. However, mumps can be deceptive when it remains unilateral and the swelling is not strictly confined to the parotid. In this case of mumps (a, b), the swelling involved the side of the face, causing lid and facial edema. Unilateral total deafness is a complication of mumps. Fig. 5.12 Mumps. Acute viral parotitis is a common infection, and the diagnosis is usually obvious. Well-defined tender swelling of the parotid gland, first on one side and shortly after on the other, with associated trismus and malaise, are characteristic. However, mumps can be deceptive when it remains unilateral and the swelling is not strictly confined to the parotid. In this case of mumps (a, b), the swelling involved the side of the face, causing lid and...

Drosophila melanogaster

The zebrafish system has proven useful for the identification of novel drug targets by the application of classical forward genetics (or knockdown by morpholino) to identify mutations that mimic human disease phenotypes. The obvious advantage of zebrafish over other model systems is that organ-level and vertebrate-specific diseases can be modeled. The first demonstration of the utility of zebrafish for identification of human disease genes was in the area of anemia the iron transporter ferroportin 1 was originally discovered in zebrafish116 and subsequently found to be mutated in the hereditary iron overload disease hemochromatosis.117,118 Models for polycystic kidney disease,119-121 melanoma,122 and various leukemias123-125 have been developed. Complex nervous system and sensory defects have been modeled in zebrafish, including neurodegeneration caused by heterologous expression of mutant tau126 and various forms of blindness and deafness.127,128

Developmental Neurobiology of the Ear Current Status and Future Directions

The final two chapters in this volume examine an exciting emerging field in inner ear development, the development of the stereociliary bundle located on all mechanosensory hair cells. In Chapter 6, Bryant, Forge, and Richardson describe the morphological process of hair-cell differentiation, including the development of the stereociliary bundle, while in Chapter 7, Hertzano and Avra-ham review insights into the development of the inner ear that have been obtained through the identification of genetic mutations that underly human non-syndromic and syndromic deafness. Surprisingly, a number of these mutations have direct effects on the development of stereociliary bundles. Although the stereociliary bundle is comprised of modified microvilli that are not dissimilar from the microvilli located on many developing and mature epithelial cells, the striking arrangement of these cells into a staircase pattern with a specific plane of polarization, and the exquisite sensitivity of this...

Blepharophimosis Syndrome

Blepharophimosis Ptosis Epicanthus

Intelligence is not affected and there are no associated systemic abnormalities. The condition is always bilateral and symmetrical. Blepharophimosis is also seen as a part of other syndromes including Michels syndrome (blepharophimosis plus cleft lip and-palate, and mental retardation), Carnevale syndrome (blepharophimosis plus abnormal ears, strabismus, and umbilical diastasis), OSA syndrome (blepharophimosis plus humeroradial synostosis and spinal abnormalities), van den Ende-Gupta syndrome (blepharophimosis plus arachnodactyly and congenital contractures), the Schwartz-Jampel syndrome (blepharophimosis plus ptosis and blepharospasm), and Ohdo syndrome (blepharophimosis plus mental retardation, dental hypoplasia, and partial deafness).

Action of the Thyroid Hormones

That the TR 1 isoform of the receptor is the one which promotes, via T3, the vital development of the central nervous system in the fetus, the neonate and the child in combination with the a1 receptor 39, 40 . It is of interest that the deafness that exists in the TRp 1 knockout rat also exists in endemic cretinism in some patients with resistance to thyroid hormones due to lack of the TR 1 gene 41 .

Otoscopy Facial Nerve

Fungal Ear Infection Otomycosis

While otoscopy alone can establish the diagnosis in some cases, parameters such as history, or audiolog-ical and neuroradiological evaluation are required in others. An important aspect of this atlas is that it juxtaposes, when appropriate, the clinical picture, radiological diagnosis, and intraoperative findings with the otoscopic findings of the patient. Needless to say, every patient should be considered as a whole and in some particular cases, the otoscopic findings might only be the tip of the iceberg. Otalgia, otorrhea, and granulations in the external auditory canal are manifestations of otitis externa, but when they persist, particularly in the elderly, they should arouse suspicion of malignancy. Otitis media with effusion can be a simple disease when seen in children, whereas unilateral persistent otitis media with effusion in an adult may be the only sign of a nasopharyngeal carcinoma. A small attic perforation in the presence of facial nerve paralysis and sensorineural...

NOC Anxiety Control

Describe hearing loss (e.g., do things sound muffled, or is there no sound in the affected ear ). hearing loss is temporary and will resolve with treatment. Provide information about OM and answer any questions. Describe action of drug that will resolve OM and restore hearing. Complications of OM may include conductive hearing loss or a perforated tympanic membrane.

Risk Of Congenital Infection

For infants with congenital infection, the risk of congenital anomalies is also closely associated with the gestational age of the fetus at the time of maternal infection. The risk for defects in neonates infected during the first trimester of pregnancy is very high. As many as 85 of neonates of mothers with infection during the first 4 weeks of gestation have congenital anomalies, but the risk falls to 20-30 during the second month of gestation and 5 during the third month (13). Infection after the 16th week of gestation rarely results in congenital defects in the infant that are detectable at birth (3). A number of abnormalities may not be present or not detected at birth but can present months to years later (10). These include deafness, ocular abnormalities, endocrinopa-thies, and central nervous system disease.

Discharge And Home Healthcare Guidelines

Most patients recover completely, but approximately 12 experience complications that include postherpetic neuralgia (PHN), uveitis, motor deficits, infection, and systemic involvement such as meningoencephalitis, pneumonia, deafness, or widespread dissemination. In some patients, the scars are permanent.

Molecular Basis of Neurosensory Cell Fate Specification

In contrast to vestibular primary sensory neurons, cochlea (spiral) primary sensory neurons express Gata3 (Karis et al. 2001 Lawoko-Kerali et al. 2002). GATA factors, including grain and, presumably, its vertebrate homolog GATA3, (Brown and Castelli-Gair Hombria 2000 Bertrand et al. 2002 Karis et al. 2001) can interact with bHLH dimers for transcriptional regulation. These genes may be directly involved in specific aspects of cochlear and vestibular fate determination via regulation of bHLH gene transcription. Unfortunately, most Gata3 mutations result in early lethality before hair cells differentiate. Nevertheless it is important to note that a human GATA3 mutation exists and causes deafness (Van Esch et al. 2000). In summary, the genes that regulate formation of the neurosensory aspects of ear development are beginning to emerge and their functions have been tested in specific mutant mice. While this line of research has provided dramatic breakthroughs in our understanding of this...

Predisposition to papillomaviruses epidermodysplasia verruciformis

Inherited deafness (Kurima et al 2002). EVER1 TMC6 and EVER2 TMC8 are normally expressed in the endoplasmic reticulum of keratinocytes (Ramoz et al 2002). Despite recent progress, many questions about the function of EVER1 TMC6 and EVER2 TMC8 and the role of their mutant alleles in the pathogenesis of EV remain unresolved. Patients with severe combined immunodeficiency (SCID) caused by yc and JAK3 deficiencies, but not SCID patients bearing other molecular defects, were recently shown to develop an EV-like clinical syndrome, despite successful haematopoietic stem cell transplantation (Laffort et al 2004). This probably reflects impaired expression of the two genes in keratinocytes, and possibly the impaired binding of their products with those of EVER1 and EVER2 (Laffort et al 2004). The in vitro) study of these genes in keratinocytes and the identification of new genetic aetiologies of EV should provide insight into protective immunity to papillomaviruses and the pathogenesis of...

Summary and Conclusions

Future directions will have to identify progenitor populations, hopefully with the capacity to generate both sensory neurons and hair cells, to restore senso-rineural hearing loss. Using the known guidance properties of neurotrophins, in particular of BDNF, could allow for a more directed growth of nerve fibers toward cochlear implants. This way the basic science gain could be translated into clinical applications beneficial for a growing number of patients suffering hearing loss.

Inner Ear Visible In

Classification Tympanic Atelectatic

In cases that do not resolve despite proper medical treatment (nasal and systemic decongestants, mucolytics, and antibiotics) or in cases with persistent conductive hearing loss (see Figs. 4.1 and 4.2), the insertion of a ventilation tube is indicated. In children, adenoidectomy is also performed. Surgery aims at alleviating the conductive hearing loss avoiding the sequelae of otitis media with effusion. Sequelae include recurrent otitis media, tympanosclerosis, adhesive otitis media, retraction pockets with eventual cholesteatoma formation, and in some long-standing cases the formation of cholesterol granuloma (see Chapter 5). In this chapter, some typical cases of otitis media with effusion will be shown. For the surgical Figure 4.1 Conductive hearing loss. Bone conduction is normal. Air conduction is on an average of 35 dB. Figure 4.1 Conductive hearing loss. Bone conduction is normal. Air conduction is on an average of 35 dB. Figure 4.7 Right ear. Seromucoid effusion in the middle...

Audiogram Of Otitis Media

Tympanic Membrane Normal

Figure 6.23 Audiogram of the same case showing conductive hearing loss. Figure 6.23 Audiogram of the same case showing conductive hearing loss. In grade I, II, and III atelectasis, a long-term ventilation tube is usually inserted to prevent further retraction of the tympanic membrane. However, in cases with marked conductive hearing loss that denotes erosion of the incus or the superstructure of the stapes, ossiculoplasty is performed after extraction and sculpturing of the eroded incus or using a homologous incus. A large disk of tragal cartilage is used to reinforce the tympanic membrane. Indications for surgery in adhesive otitis media include cases with tympanic membrane perforation (grade V according to Sade 1979), with or without polypi, granulation or otorrhea, those cases with a large infected retraction pocket causing frequent otorrhea, or those with conductive hearing loss due to ossicular chain erosion. In all these cases a tym-panoplasty is performed using a postauricular...

Tympanic Membrane Perforation

Retraction Pocket

Acquired cholesteatoma of the middle ear can be caused by invasion of the skin of the external auditory canal into the middle ear through a marginal perforation. It can also originate from a epitympanic retraction pocket that becomes so deep that keratin debris can no longer be expelled, leading to their accumulation and subsequent cholesteatoma formation. Such retraction pockets can remain asymptomatic until they become infected, resulting in otorrhea and hearing loss. In other cases, the only symptom might be progressive hearing loss due to erosion of the ossicular chain by the developing cholesteatoma. Fetid otorrhea and hearing loss are the main complaints in cholesteatoma. In addition, complicated cases can manifest with vertigo and or facial nerve paralysis. Vertigo occurs as a result of labyrinthine fistula, which is most commonly located in the lateral semicircular canal. Facial paralysis can be caused by pressure of the cholesteatoma sac or neuritis.

Clinical manifestation

Zoster oticus (geniculate zoster, zoster auris, Ramsay-Hunt syndrome, Hunt syndrome) Meniere disease, Bell palsy, cer-brovascular accident or abscess of the ear beginning with otalgia and herpetiform vesicles on the external ear canal, with or without features of facial paralysis, resulting from facial nerve involvement, auditory symptoms (e.g., deafness), and vestibular symptoms

Cribriform Plate Infection

Subdural Empyema Pathogenesis

Kernigs sign (resistance to passive raising of leg with extended knee) and Brudzinski's sign (involuntary leg flexion on passive flexion of the neck) are signs of meningeal involvement. Painful neck stiffness is due to (lepto)meningeal irritation by infectious meningitis, septicemia, subarachnoid hemorrhage, neoplastic meningitis, or other causes. Isolated neck stiffness not caused by meningitis (meningism) may be due to cervical disorders such as arthrosis, fracture, intervertebral disk herniation, tumor, or extrapyramidal rigidity. Papilledema is usually absent when present, it indicates intracranial hypertension (p. 158). Encephalitic syndrome is characterized by headache and fever, sometimes accompanied by epileptic seizures (often focal), focal signs (cranial nerves deficits, especially of CN III, IV, VI, and VII aphasia, hemiparesis, hemianopsia, ataxia, choreoathetosis), behavioral changes, and impairment of consciousness (restlessness, irritability, confusion,...

Review of auditory mechanisms

In reality, the sensorineural hearing loss that is treated by hearing aids and MEIHDs is the same pathology as is indicated for cochlear implants loss of inner ear hair cells. In moderate to severe hearing loss, the reduction in hair cell numbers is such that sensitivity to sounds has been reduced amplified sounds or ossicular movements recruit a greater portion of the remaining hair cells and socially adequate hearing is restored. In profound hearing loss, the hair cell population is so low that neither acoustic amplification by hearing aids nor mechanical overdrive by MEIHDs can provide adequate restoration of hearing. Direct stimulation of spiral ganglion cells by passing electrical current across these nerves effectively bypasses the hair cell transducers and initiates action potentials to the first relay in the auditory pathway to the brain, the cochlear nucleus. In yet a third form of neural hearing loss, the Vlllth cranial nerve, the statoacoustic nerve, has been damaged or...

Types of implantable hearing devices

The four categories of implantable hearing devices (IHD) are classified according to the types of hearing losses in individuals. These categories are bone conductors, middle ear implants, cochlear implants, and auditory brainstem implants. When conductive hearing loss is present, a bone conduction IHD such as the bone-anchored hearing apparatus (BAHA Entific Medical Systems, Gothenberg, Sweden) seeks to restore hearing by vibratory conduction through the skull to the inner ear. Bone conduction devices are intended for use with conductive hearing loss, whereas the remainder of the IHDs seek to restore hearing due to sensorineural hearing loss. Sensory hearing impairment is addressed by MEIHDs and the cochlear implant. MEIHDs treat moderate, moderate-to-severe, and severe hearing losses by amplification of sound signals through implanted transducers. These amplification mechanisms utilize either piezoelectric crystals or electromagnetic transducers where greater displacement of the...

Wireless Implantable Microsystem

Silicon micromachined electrode arrays permit the long-term monitoring of neural activity in vivo as well as the insertion of electronic signals into neural ensembles or networks at the cellular level. Such electrode arrays are facilitating significant advances in the understanding of the nervous system, and merged with on-chip circuitry, signal processing, microfluid-ics, and wireless interfaces, they are forming the basis for a family of neural prostheses for the possible treatment of disorders such as blindness, deafness, paralysis, severe epilepsy, and Parkinson's disease.

Primary Nursing Diagnosis

Other Drugs Adjunct corticosteroid therapy has been reported to decrease the inflammatory process and decrease incidence of hearing loss but is controversial. Vaccinations exist for meningococcal, pneumococcal, and hemophilic meningitis, and the prophylaxis for persons exposed to meningococcal meningitis is rifampin.

Facioscapulohumeral Muscular Dystrophy

Facioscapulohumeral muscular dystrophy (FSHD) is a dominantly inherited muscular dystrophy affecting about 1 in 20,000 individuals. The weakness is not restricted to areas named in the disease name weakness may occur in hip girdle, ankle dorsiflexors, and occasionally oropharynx. Extramuscular manifestations of this slowly progressing myopathy may include hearing loss and retinal vasculopathy. All patients with a confirmed diagnosis of FSHD carry a chromosomal rearrangement within the subtelomeric region of chromosome 4q (4q35). This subtelomeric region is composed mainly of a polymorphic repeat structure consisting of 3.3-kb repeated elements (D4Z4). The number of repeat units varies from 10 to more than 100 in the population, and, in patients with FSHD, an allele of 1 to 10 residual units is observed because of the deletion of an integral number of these units.

Factors Influencing Spoken Language Outcomes in Children following Early Cochlear Implantation

Development of spoken language is an objective of virtually all English-based educational programs for children who are deaf or hard of hearing. The primary goal of pediatric cochlear implantation is to provide critical speech information to the child's auditory system and brain to maximize the chances of developing spoken language. Cochlear implants have the potential to accomplish for profoundly deaf children what the electronic hearing aid made possible for hard of hearing children more than 50 years ago. Though the cochlear implant does not allow for hearing of the same quality as that experienced by persons without a hearing loss, it nonetheless has revolutionized the experience of spoken language acquisition for deaf children. However, the variability in performance remains quite high, with limited explanation as to the reasons for good and poor outcomes. Evaluating the success of cochlear implantation requires careful consideration of intervening variables, the characteristics...

Clinical Uses Of Electrical Stimulation

Cochlear implants stimulate spinal ganglion cells of the auditory nerves, bypassing nonfunctional hair cells to restore limited hearing in some types of deafness. The cochlear implant system really consists of an implanted stimulator connected to an electrode array inserted in the cochlea and an external speech processor that codes the speech into stimulation patterns that can be translated back into sounds by the brain. The external speech processor also powers the implant via an inductive energy transfer link. Cochlear implants are now common and provide substantial benefits to many profoundly deafened children and adults. Benefits vary by person and range from increased perception of environmental sounds to the ability to use a telephone.

Pregnancy and Lactation

Therapy with chloroquine during pregnancy is discussed controversially. Chloro-quine was associated with sensorineural hearing loss, blindness, malformations, abortion, and, in vitro, chromosomal damage. The frequency of these events was calculated with 1.2 -3.3 (control group < 1 ). On the other hand, there are several reports on healthy newborns after chloroquine therapy of the mother. It is agreed that malaria prophylaxis (2x500 mg week) should be continued during pregnancy. In the case of a planned conception, however, it was recommended to discontinue chloro-quine use in time. Here the potential risk of the treated disease, like activation of SLE (Wechsler et al. 1999),has tobe weighed individually against the possible chloroquine side effects (Parke and West 1996). Recently, it was not recommended any longer to discontinue antimalarials in pregnant patients with SLE (Borden and Parke 2001). In a series of 21 newborns of mothers using chloroquine (daily dose, 332 mg) or...

Sensory Coding Properties Reflect Synapse Rearrangement

A similar finding is obtained in cats that were deafened by damaging the inner hair cells, and then stimulated electrically within the cochlea. This is a highly relevant experimental model because children with profound hearing loss are now routinely implanted with cochlear prostheses, and they hear the acoustic world via electrical stimulation of their cochlea

Sensory Deficits Introduction

Auditory disorders are classified as conductive, sensorineural or mixed conductive-sensorineural hearing loss. Causes include damage to the inner ear structures or the auditory nerve from congenital defects, infection, ototoxic drugs, long-term excessive exposure to noises (sensorineural) or middle ear infection such as otitis media (conductive).

Homeostasis The More Things Change The More They Stay The Same

What is the evidence that synaptic strength is adjusted by a similar homeostatic mechanism in vivo To answer this question, direct measures of EPSP and IPSP amplitudes have been made in a brain slice preparation following a period of sensory deprivation (e.g., blindness or deafness). When gerbils are surgically deafened before they would first experience sound, compensatory responses are observed for both excitatory and inhibitory synapses within the inferior colliculus. Inhibitory synaptic conductance declines, and the inhibitory reversal potential depolarizes. In contrast, afferent-evoked excitatory synaptic responses become larger and longer in duration (Vale and Sanes, 2000, 2002). Interestingly, the inhibitory reversal potential appears to become depolarized because chloride transport is downregulated (Vale et al., 2003). Similar observations have been made in the cortex. During normal development of the rat visual cortex, the amplitude of miniature EPSCs declines during the...

Diagnostic Procedures

The following procedures are used to test for hearing loss and to diagnose or rule out certain possible underlying causes of hearing loss Audiometry. This is the most commonly used hearing test. The first part measures how well you can hear sounds conducted through the air and indicates the condition of your overall hearing. The test is usually administered in a soundproof room using a machine called an audiometer. Through headphones, you listen to a series of sound tones that range from high to low, one tone at a time. Each tone begins at an easily audible sound level. You indicate with a prearranged signal when you hear the tone. The sound level decreases gradually until you are no longer able to hear the tone this point is your hearing threshold for that frequency. The second part of the test measures how well you can hear sounds conducted through your head and indicates whether your hearing loss is conductive or sensorineural (see page 397). The procedure is the same as for the...

POU Transcription Factors

As described below, not only is the expression strong during early development of the embryo, but the Pou3f4 mouse mutants have structural abnormalities in their auditory system during development. Mutations in the POU3F4 transcription factor are associated with the DFN3 locus on chromosome X (de Kok et al. 1995). The DFN3 phenotype is variable, and at the very least it is characterized by profound sensorineural hearing loss but is also often associated with conductive hearing loss and with stapes fixation. Furthermore, as DFN3 maps to chromosome Xq21 in the region containing mental retardation and choroideremia, some patients with POU3F4 mutations have additional symptoms due to deletions of larger portions of the chromosome. Both point mutations in the POU3F4 gene, as well as larger deletions, duplications, and inversions of the Xq21 region are associated with the DFN3 phenotype. The deletions account for a little over half of DFN3 mutations and many of them do not...

Rocky Mountain Spotted Fever

Rickettsia rickettsii This is transmitted via contact with the wood tick, the dog tick, or the Lone Star tick, with an overall incidence of 0.2-0.5 cases per 100000 population. The usual neurological features consist of headache, neck stiffness, altered sensorium, and convulsions. Other neurological abnormalities include ataxia, aphasia, neural hearing loss, and papilledema. The neuro-pathological findings consist of cerebral edema, perivascular and meningeal lymphocytic infiltration, and extensive necrotizing vasculitis

Gap Junction Proteins

One of the most dramatic discoveries in the field of hereditary hearing loss in recent years has been the large number of connexin mutations. Gap junction proteins encode the connexins, a component of connexons that allow molecules to pass from cell to cell. Two networks of gap junctions, the epithelial cell system and the connective tissue cell system, are functional in the cochlea (Kumar and Gilula 1996). Three connexins have been implicated in deafness connexin 26 (GJB2), accountable for approximately 30 of childhood deafness (Denoyelle et al. 1997 Cohn et al. 1999) connexin 30 (GJB6) in NSHL (Lerer et al. 2001 del Castillo et al. 2002) and connexin 31 (GJB3) both in NSHL (Xia et al. 1998 Liu et al. 2000) and peripheral neuropathy and HL (Lopez-Bigas et al. 2001).

Conduction of Sound Sound Sensors

Kno Membvrana Tympani

In the middle ear, the malleus, incus and stapes conduct the vibrations of the tympanic membrane to the oval window. Their job is to conduct the sound from the low wave resistance impedance in air to the high resistance in fluid with as little loss of energy as possible. This impedance transformation occurs at f < 2400 Hz and is based on a 22-fold pressure amplification (tympanic membrane area oval window area is 17 1, and leverage arm action of the auditory ossicles amplifies force by a factor of 1.3). Impairment of impedance transforming capacity due, e.g., to destruction of the ossicles, causes roughly 20 dB of hearing loss (conduction deafness). Bone conduction. Sound sets the skull in vibration, and these bone-borne vibrations are conducted directly to the cochlea. Bone conduction is fairly insignificant for physiological function, but is useful for testing the hearing. In Weber's test, a vibrating tuning fork (a1) is placed in the middle of the head. A person with normal...

Histopathology of the Inner Ear Relevant to Cochlear Implantation

The most common forms of severe hearing loss and deafness are related to morphological changes in the cochlea. Many individuals with such forms of hearing disorders have received cochlear implants. It has been assumed that preservation of spiral ganglion cells is important for success of cochlear implants. Preservation of ganglion cells is negatively correlated with the duration of the hearing loss. It has, however, not been possible to reveal a relationship between the degree of survival of spiral ganglion cells and performance of cochlear implants. It is important to understand the histopathological changes that follow cochlear implantation. Insertion of cochlear implants may cause trauma to the basilar membrane, the spiral lamina, and the spiral ligament. Rupture of the basilar membrane may occur. Over time, new bone forms at the cochleostomy and along the implant track. Further investigation is necessary to evaluate the causes of variability of behavioral measures of performance....

Surgical Myringotomy with Tube Insertion Age 017

Tubes, particularly in infants and young children, because the amount and stiffness of their cartilage is less than that of older children and adults. Eustachian tube obstruction leads to negative middle ear pressure and a sterile MEE. Drainage of the effusion is inhibited by impaired mucociliary action and sustained negative pressure. Contamination of the middle ear may occur from nasopharyngeal secretions and lead to infection. Because infants and young children have a shorter eustachian tube than older children, it makes them more susceptible to reflux of nasopharyngeal secretions into the middle ear and development of infection. Other predisposing factors include upper respiratory infections, allergies, Down syndrome, bottle propping during feedings, daycare attendance, and parental smoking. Complications include persistent AOM, tympanic membrane perforation, mastoiditis, hearing loss for several months, speech delay, and cerebral thrombophlebitis.

Volume Preface

The final two chapters in this volume examine an exciting emerging field in inner ear development, the development of the stereociliary bundle located on all mechanosensory hair cells. In Chapter 6, Bryant, Forge, and Richardson describe the morphological process of hair cell differentiation, including the development of the stereociliary bundle, while in Chapter 7, Hertzano and Avra-ham review insights into the development of the inner ear that have been obtained through the identification of genetic mutations that underlie human nonsyndromic and syndromic deafness.

Charge Syndrome

Originally described in the early 1980s, CHARGE is the acronym for coloboma, heart disease, atresia choanae, retarded growth and retarded development and or central nervous system anomalies, genital hypoplasia, and ear anomalies and or deafness. CHARGE is clinically diagnosed, so the frequency and certainty will depend on who is doing the evaluation. With the establishment of specific etiology or etiologies for CHARGE, the diagnostic criteria will likely improve. Most cases of CHARGE syndrome appear to be new mutations the loci for many has been mapped to chromosome 8q12. The incidence is about 1 10,000 births, but high infantile mortality rates likely obscure the true incidence of this syndrome.

Prenatal Diagnosis

In addition, erroneous assumptions about the significance of IgG and IgM antibody levels could lead to provision of incorrect information regarding risk to the fetus. Even when a prenatal diagnosis of congenital CMV infection is accomplished, the only possible clinical recourse involves the decision of whether to terminate the pregnancy. Although CMV is a leading cause of brain disease and hearing loss in the United States, the outcome of congenital CMV infection is highly variable, thus limiting the value of prenatal diagnosis.

Hemifacial Spasm

Spasmmuscle Disorder Images

TREATMENT Anticonvulsants, such as carbamazepine, may be helpful in some cases, as are the short-acting benzodiazepines. But these are useful in only about one-fourth of cases with mild symptoms. The most effective treatment currently available is chemodenervation with botulinum toxin. The latter blocks neuromuscular transmission by preventing degranulation of acetylcholine at peripheral cholinergic nerve endings. The duration of effect is typically three to four months. Complications include transient ptosis, mouth droop, and other muscle weaknesses. Microvascular decompression of the seventh nerve root where it exits the brain stem is an effective surgical treatment in most cases, with about 70 to 89 of cases showing complete relief of spasms. Potential complications include hearing impairment and CSF leak, and the mortality rate is reported at 0.3 to 0.6 . Monitoring of abnormal muscle response intraoperatively may enhance the final result.

Auditory Pathway

Hearing Pathway Blood Vessel

Axons of the cochlear nerve originating in the cochlear apex and base terminate in the anterior and posterior cochlear nuclei, respectively. These nuclei contain the second neurons of the auditory pathway. Fibers from the posterior cochlear nucleus decussate in the floor of the fourth ventricle, then ascend to enter the lateral lemniscus and synapse in the inferior colliculus (third neuron). The inferior colliculus projects to the medial geniculate body (fourth neuron), which, in turn, projects via the acoustic radiation to the auditory cortex. The acoustic radiation passes below the thalamus and runs in the posterior limb of the internal capsule. Fibers from the anterior cochlear nucleus also decussate, mainly in the trapezoid body, and synapse onto the next (third) neuron in the olivary nucleus or the nucleus of the lateral lemniscus. This branch of the auditory pathway then continues through the lateral lemniscus to the inferior colliculus and onward through the acoustic radiation...

Cochlear implants

Deafness can be the result of many causes. Deafness that is the result of defects in the outer and middle ear, but not further up in the sound sensing system, can be corrected by a hearing aid. Cochlear implants3233 are used for those people who do not have sensory hair cells and their hearing loss cannot be corrected by the use of a hearing aid. The cochlear implant electrodes stimulate the auditory nerve. For people with a defect in auditory nerve, auditory brainstem implants (ABIs) are required however, ABIs are still in the developmental stages. Initially, cochlear implants were developed to imitate the lost function of the cochlea. Understanding of sound information processing by the cochlea and brain has contributed to the enhancement in the implant effectiveness. For example, it has been found that the sound-sensing cells are organized in the cochlea tonotopically. The apex of the Several factors have been found to affect the effectiveness of the implant. For example, people...

Cognition

This is a crucial part of the evaluation and needs to be done in a systematic and comprehensive and non-threatening manner. The initial aspects have to do with observation as described above in the section on general appearance, manner, andattitude,observingfor level of alertness, consciousness, confusion, fluctuation, somnolence, or stupor. Careful observation may reveal perseveration on words, numbers, or actions. Perseveration may be evident in the absence of hearing impairment when the patient responds to a prior question more than one time as if he or she had not heard the following question. Specific questions as to orientation can be approached in an ego-supportive manner and can be asked as part of the routine. Memory is best tested by observing the patient's ability to provide his or her medical history in an organized manner and asking direct and specific questions about onset, course, and treatments. If a patient spontaneously reveals that memory is a problem, this lead can...

Acknowledgements

The TMC gene family encoding transmembrane proteins. BMC Genomics 4 24 Kurima K, Peters LM, Yang Y et al 2002 Dominant and recessive deafness caused by mutations of a novel gene, TMC1, required for cochlear hair-cell function. Nat Genet 30 277-284 Kurima K, Yang Y, Sorber K, Griffith AJ 2003 Characterization of the transmembrane channellike (TMC) gene family functional clues from hearing loss and epidermodysplasia verruciformis. Genomics 82 300-308 Laffort C, Le Deist F, Favre M et al 2004 Severe cutaneous papillomavirus disease after haemo-poietic stem-cell transplantation in patients with severe combined immune deficiency caused by common gamma cytokine receptor subunit or JAK-3 deficiency. Lancet 363 2051-2054 Latour S, Veillette A 2004 The SAP family of adaptors in immune regulation. Semin Immunol 16 409-419

Myringostapedopexy

Air Fluid Levels Ear

Adhesive otitis media or grade IV atelectasis associated with a mild epitympanic retraction pocket. The thin and atrophic tympanic membrane completely covers the promontory. The tympanic membrane retraction has caused erosion of the long process of the incus with a subsequent spontaneous myringostapedopexy As the patient does not complain of hearing loss, surgery is not indicated. Figure 6.21 Left ear. Posterior retraction pocket. The tympanic membrane remains adherent to the stapes' head even after Valsalva maneuver (myringostapedopexy). The remaining part of the tympanic membrane is thick and shows tympanosclerosis. Audiometry revealed normal hearing. Cases with myringostapedopexy generally have good hearing therefore, surgery is not indicated except if conductive hearing loss develops and or a posterior retraction pocket is associated with frequent otorrhea. Surgery varies from simple myringoplasty (when the tympanic membrane needs reinforcement) to...

History

The syndrome of familial profound congenital hearing loss associated with large, multinodular goiter was first described by Vauhan Pendred 1 in 1896 (OMIM 274600). From its initial description until 1996, a large number of articles were written describing novel cases and suggesting possible pathophysiologic mechanisms for the syndrome. However, all of these were based on speculation, since the precise cause of the syndrome remained unknown for 100 years. Then, in 1996, using linkage analysis, 2 groups independently identified the genetic locus responsible for the syndrome 2, 3 . The discovery of the precise gene mutated in Pendred syndrome, only 1 year later, opened a new phase in the history of the syndrome 4 . Pendred syndrome is caused by loss-of-function mutations in the novel protein called pendrin, which is encoded by the gene SLC26A4 (PDS). Pendrin functions as an anion transporter and is expressed in the thyroid, the inner ear, the kidney and the placenta. The precise...

Molecular Genetics

More than 100 different PDS (SLC26A4) mutations have been reported in patients with Pendred syndrome and nonsyndromic deafness (see below) (fig. 4). Most of these are seen in only a single family, although 4 specific mutations (E384G, L236P, T416P, and 1001 + 1G) are commonly seen, and are estimated to be responsible for 50-60 of the Pendred syndrome cases in the Caucasian population 26, 27 . Haplotype analysis suggests that these are founder mutations in the Northern European Caucasian population and not mutation hot spots. Another founder mutation, a single base deletion causing a frame shift and truncated protein (1220delt), was identified in a large Bedouin tribe from Northern Israel with more than 35 patients diagnosed with Pendred syndrome.

Tympanic Membrane

Tympanic Membrane Retraction Grade

Grade I atelectasis with the malleus slightly medialized. An epitympanic retraction pocket is also seen. Middle ear effusion with yellowish color can be appreciated. Pure tone audiogram revealed a 40-dB conductive hearing loss (Fig. 6.6), whereas the tympanogram was type B, i.e., typical of middle ear effusion (Fig. 6.7). In this case, the insertion of a ventilation tube is indicated to avoid further retraction of the tympanic membrane, to aerate the middle ear, and to improve hearing. Figure 6.6 Audiogram of the same case showing a 40-dB conductive hearing loss.

Conclusion

LCMV infection should be considered as a potential etiology of congenital infection in babies with CNS disease (micro- or macrocephaly, hydrocephalus) and chorioretini-tis. The disease most closely resembles congenital toxoplasmosis or CMV infection in babies with the exception that LCMV-infected newborns rarely exhibit hepatosple-nomegaly or hearing loss. Diagnosis of congenital LCMV infection is based on compatible clinical findings, serologic evidence of LCMV infection (IgM and IgG against LCMV), and negative diagnostic studies for other congenital infections (CMV culture, toxoplasmosis serology, syphilis, enterovirus, rubella, and herpes simplex virus).

Clinical Syndrome

The hallmark of Pendred syndrome is the combination of sensorineural deafness and goiter in the presence of a positive perchlorate discharge test. The clinical manifestations of this syndrome can be highly variable between affected families and even within the same family 5 . The true incidence of the syndrome is not known, and depends in part on whether it is defined on a purely clinical basis, or on a genetic basis. The incidence of congenital deafness has been reported to be between 1 1,000 and 1 2,000, and 1-8 of patients with congenital hearing loss are thought to have Pendred syndrome 6, 7 . This suggests that the incidence of Pendred syndrome may range from 1 12,500 to 1 200,000. However, this estimate is based on data collected long before the genetic etiology of Pendred syndrome was known. New studies are needed to determine what percentage of patients with congenital hearing loss have mutations in the PDS gene, and what percentage of these have complete syndrome (see below)....

Myosins

Myosins were among the first group of proteins found to be associated with hereditary hearing loss. These motor molecules are divided into 17 classes based on analysis of their motor and tail domains and numbered in the order in which they were discovered (Myosin Homepage, http www.mrc-lmb.cam.ac. uk myosin myosin.html). The myosins are comprised of a heavy chain with a conserved approximately 80 kDa catalytic domain (the head or motor domain), and most are followed by an a-helical light chain-binding region (the neck region) (Sellars 1999). All myosins contain an actin-binding domain and an ATP-binding domain in their head region, allowing them to move along actin filaments. Most myosins contain a C-terminal tail and in some cases, an N-terminal extension as well. The tail domains diverge from one another between myosin classes and are believed to confer the function of each different myosin. Following is a number of myosins that when mutated, were found to compromise both auditory...

Myo7adfnb3dfna11

Myosin VIIA mutations were first detected in patients with a syndromic form of HL, USH1B, associated with retinitis pigmentosa (RP) (Weil et al. 1995). The shaker 1 (shl) mouse was instrumental in the elucidation of the human gene (Gibson et al. 1995). Two years later, mutations associated with NSHL, DFNB2, and DFNA11 were discovered, although much rarer than those contributing to USH1B (Liu et al. 1997a, b). DFNB2 is an autosomal recessive form of NSHL, identified in Chinese and Tunisian families. The mutation in the Tunisian family is a G1797A missense, changing a methionine to isoleucine at the end of exon 15, leading to a decrease in splicing efficiency. Affected DFNA11 family members from Japan have moderate progressive sensorineural hearing loss, with an in-frame nine-base-pair deletion that results in the loss of three amino acids (Tamagawa et al. 2002). This deletion occurs in the coiled-coiled region of the myosin VIIA tail and thus is predicted to prevent dimerization of...