Facioscapulohumeral muscular dystrophy and accompanying hearing loss

Facioscapulohumeral (FSH) muscular dystrophy (MD) is an autosomal dominant condition marked by muscle weakness primarily in the face, shoulder girdle, and legs. Intelligence and life span are normal and the spectrum of disability is broad. Flaccid dysarthria results from the facial muscle paralysis. A pair of siblings had FSH MD that was accompanied by the unusual finding of sensorineural hearing loss. Speech and audiologic aspects are presented and etiologic explanations are suggested. Careful audiologic evaluation of all patients with FSH MD is advised so that sensorineural hearing loss can be ruled out.     

Hearing threshold estimation in infants using auditory steady-state responses

Successful early intervention in children with permanent hearing loss requires assessment techniques that can accurately reflect the behavioral audiogram in infancy. This retrospective study compared auditory steady-state response (ASSR) findings from subjects tested in the first three months of life with subsequently obtained behavioral hearing levels. ASSR audiograms were established using amplitude and frequency modulated tones at octave frequencies (500 Hz to 4 kHz). Results obtained from 575 subjects including 285 with normal hearing, 271 with sensorineural hearing loss, and 19 with auditory neuropathy-type hearing loss are presented. ASSR and behavioral hearing thresholds for subjects in the normal and sensorineural groups were highly correlated, with Pearson r values exceeding 0.95 at each of the test frequencies. In contrast, ASSR thresholds in children with AN-type hearing loss did not accurately reflect the behavioral audiogram. Overall, the findings indicate that ASSR testing can offer useful insights into the hearing acuity of children tested in infancy.     

Sensorineural hearing loss and Kawasaki disease: a prospective study

PURPOSE: Kawasaki disease (KD) is an acute, self-limited vasculitis of infants and children that is now the most common cause of acquired heart disease in the pediatric age group in the United States and Japan. Reports have documented the association of acute KD with sensorineural hearing loss. To assess the prevalence of hearing loss following acute KD in a geographically and ethnically diverse population, a prospective, multicenter study of hearing loss in patients with KD was conducted. MATERIALS AND METHODS: Patients with acute KD were enrolled in 7 clinical centers and underwent a primary audiologic evaluation within 30 days of the onset of fever. Patients were subsequently reevaluated after resolution of the acute phase of the disease. A questionnaire assessing risk factors for hearing loss was also administered. RESULTS: A total of 62 patients were evaluated during the 29-month study period. At the first audiologic evaluation, 19 patients (30.6%) had sensorineural hearing loss, 6 patients (9.7%) had conductive hearing loss, 17 patients (27.4%) had normal hearing, and 20 patients (32.3%) had inconclusive studies. Overall, 2 of 36 patients (5.5%) had sensorineural hearing loss documented on their second audiologic evaluation. No risk factors for hearing loss were identified by the questionnaire. CONCLUSIONS: Transient sensorineural hearing loss (20 to 35 dB) is a frequent complication of acute KD and may be related to salicylate toxicity in some patients. Persistent sensorineural hearing loss is uncommon. Parents and primary care providers should be made aware of the potential for persistent sensorineural hearing loss following resolution of KD, but routine audiologic screening of this patient population does not appear to be warranted.     

Audiologic and otologic phenotype in children with Duane's Retraction Syndrome: A rare ophthalmologic disorder

ObjectiveThe focus of this study was to evaluate the prevalence, type, and severity of hearing impairment in patients with Duane's Retraction Syndrome and to relate these measures to patient demographics and other otologic and audiologic factors.Study designRetrospective AudGen Database analysisSettingTertiary academic referral centerPatientsPediatric patients in AudGenDB with a diagnosis of Duane's Retraction Syndrome (DRS).InterventionsAppropriate audiologic, otologic, and demographic data were recorded.Main outcome measureSeventy-nine patients (n = 79) met inclusion criteria. The first encounter with available audiometric data or the first encounter with hearing loss were documented. Audiograms were stratified by type and severity of hearing loss, and common associated medical issues were documented.Results57 children had normal hearing; 22 had hearing loss. 9 ears had pure conductive hearing loss, 1 had pure sensorineural, 14 ears had components of both, and 79 had hearing loss that could not be specified. Multivariate regression revealed episodes of chronic otitis and craniofacial anomalies are associated with worse hearing loss.ConclusionThis study presents a detailed characterization of hearing loss in patients with Duane's retraction syndrome. Conductive and sensorineural hearing loss are both prevalent among these children. Careful and early audiologic evaluation of all patients with DRS is important.     

Auditory neuropathy in childhood

Objectives: Auditory neuropathy is a recently described disorder in which patients demonstrate hearing loss for pure tones, impaired word discrimination out of proportion to pure tone loss, absent or abnormal auditory brainstem responses, and normal outer hair cell function as measured by otoacoustic emissions and cochlear microphonics. We have identified eight pediatric patients having hearing deficits that are most likely due to a neuropathy of the eighth nerve. In this study, the results of audiologic testing performed with these eight children are described. Study Design: Retrospective review of audiologic findings in eight children with auditory neuropathy. Methods. Each subject was tested with pure tone and speech audiologic testing, auditory brainstem response, and click-evoked otoacoustic emissions. Results of these tests were tabulated and summarized. Results: Pure tone audiologic testing revealed five children with upsloping sensorineural hearing loss, two with high frequency loss, and one with a mild, flat configuration. Six children demonstrated poor word discrimination scores, and the other two had fair to good word discrimination. All eight subjects had normal distortion product and transient otoacoustic emissions. All eight children demonstrated absent or marked abnormalities of brainstem auditory evoked potentials. These findings suggest that while cochlear outer hair cell function is normal, the lesion is located at the eighth nerve. Conclusions: Recent advances in otoacoustic emissions testing permit differentiation of neural deafness from sensory deafness. This paper describes the clinical presentation and audiologic findings in pediatric auditory neuropathy, as well as the recommended management of these patients. Otolaryngologists should be aware of this disorder and implications for its management, which differs from treatment of sensorineural hearing loss. Key Words: Auditory neuropathy, childhood, hearing loss, auditory brainstem response, evoked otoacoustic emissions.     

Congenital perilymphatic fistula: an overlooked diagnosis?

Congenital perilymphatic fistula (CPLF) often is the sole cause of sensorineural hearing loss in children that is both progressive and subtle and, therefore, can elude detection by physicians. A 3-year prospective study of 244 children with sensorineural hearing loss of unknown etiology demonstrated that at least 6% (15/244) had CPLF. Close audiologic monitoring and radiologic imaging of the temporal bone of children with sensorineural hearing loss are recommended to detect CPLF.     

Etiological diagnosis of bilateral, sensorineural hearing impairment in a pediatric Greek population

Early diagnosis, evaluation and treatment of childhood deafness are essential for a child's normal growth. Etiological diagnosis of hearing loss makes prevention, family scheduling and more effective therapy feasible goals. Etiological assessment of sensorineural deafness still remains difficult although recently with the progress of genetics it has become more efficient. In this retrospective study, the etiology of bilateral, sensorineural hearing loss with indication for hearing aids has been studied in 153 hearing impaired children. Etiological diagnosis was based on family and patient record, physical, audiological and laboratory examinations. Among the 94 children who completed the diagnostic protocol etiological groups revealed the following distribution: non-hereditary acquired hearing impairment was present in 36 children (38%) and hereditary was present in 44 (47%) children. The etiology remained unknown in 14 (15%) children. Non-syndromic autosomal dominant type accounted for 13 (29% of hereditary hearing loss) children, non-syndromic autosomal recessive type for 21 (48%) children and syndromic deafness for 10 (23%) children. Modern diagnostic methods, such as genetic testing, help diminish the number of cases with hearing impairment of unknown etiology, for the benefit of children who receive early and appropriate medical, audiologic, genetic and educational counseling based on the etiology of their hearing loss.     

Incidence of coexisting sensorineural hearing loss and secretory otitis media

There are currently no standard guidelines for assessing hearing in children who are evaluated for tympanostomy tubes. We describe the results of audiologic testing on 587 children, age 2 months to 17 years admitted to Pediatric Otolaryngology Department Bia?ystok for treatment of secretory otitis media. Ten children (1.7%) were found to have previously unrecognized sensorineural hearing loss. In four cases total unilateral deafness, in six others moderate to severe sensorineural bilateral hearing loss was diagnosed. Three other children referred to our clinic as sensorineural hearing loss were found to have secretory otitis media as the only or coexisting cause of deafness. Results of our study show the importance of age--appropriate hearing assessment as part of diagnostic procedure for secretory otitis media.     

Childhood pseudohypacusis

Pseudohypacusis is a condition in which a hearing loss is exhibited in the absence of any organic disease. The mainstay of diagnosis is a lack of consistency in audiological testing. It is usually easier to diagnose in children than in adults, as children are less able to produce consistently erroneous results on repeated testing. In spite of this, the diagnosis is often missed in children, probably due to a lack of awareness of the condition. We present the findings in ten children seen in the past year. Initially they had average pure tone thresholds of 51.3 db in the right ear and 51.4 db in the left ear. All of the children underwent repeat pure tone audiometry and speech audiometry. In nine cases the speech audiograms confirmed the diagnosis. In one child the speech audiogram was consistent with a mild hearing loss subsequently confirmed as a 30–40 db low frequency sensorineural hearing loss. Following a programme of close follow up and support, the pure tone thresholds returned to within normal limits in nine children and to a level consistent with the clinical impression in the child with a sensorineural loss. None of the children required brain stem evoked response audiograms to confirm the diagnosis.     

Use of the argon laser in the treatment of malleus fixation

This case demonstrates the use of the argon laser for ossicular mobilization. A preoperative audiologic evaluation revealed a severe conductive hearing loss, with a maximum air-bone gap. Since normal drilling procedures would result in a sensorineural hearing loss, the argon laser was chosen to remove a bony spur connecting the malleus to the posterior canal wall. When using the argon laser, no disarticulation of the incus and stapes is required. Postoperative audiologic evaluation revealed normal hearing sensitivity bilaterally.     

cis-platinum ototoxicity in children

Despite the recognized ototoxicity of cis-platinum, a clinical outline for the audiologic evaluation of patients receiving this drug has not been clearly defined. In a practical approach to this problem, the audiograms of 48 pediatric patients referred for monitoring during planned cis-platinum therapy were reviewed. Eleven patients tested with auditory brain-stem response (ABR) audiometry demonstrated several limitations of this modality. Fourteen children underwent initial ABR testing followed by at least two pure-tone audiograms. The remaining 23 patients had their hearing evaluated by pure-tone audiometry only. Various factors such as patient age, cis-platinum dosage, and cranial radiation exposure were analyzed for apparent effect. Younger patients tended to be more susceptible to audiologic changes with the administration of cis-platinum. The proportion of patients who demonstrated a hearing loss increased with successive dosing as did the severity of the hearing loss. Prior exposure to cranial radiation was strongly linked to the development of hearing loss following cis-platinum therapy. Guidelines are presented regarding the use of clinical audiometry in the screening of these pediatric oncology patients.     

Complete evaluation of the child identified as a poor listener.

With increasing awareness among educators of the importance of early identification of hearing impairment, growing numbers of children are being referred for evaluation when a teacher or day care supervisor perceives that a child is having difficulty listening. Some children who manifest difficulty listening in a pre-school play group or the classroom may have conductive or sensorineural hearing loss, while others have normal hearing with an underlying and yet-to-be-detected behavioral or psychoeducational disorder. This report presents suggestions for evaluation of the child referred for difficulty listening. The otologist should consider that a child may have an attention deficit disorder when results of initial audiologic assessment indicate there is no hearing loss or when the degree of hearing loss appears to be small in relation to the degree of inattentiveness that has been observed. The features of Attention-deficit Hyperactivity Disorder (ADHD) and Specific Developmental Disorder (SDD) are described, and illustrative case studies are presented. Clues to diagnosis are provided and a distinction between overlapping disorders is made.     

Epidemiology of hearing loss in childhood

During a 20-year period, half a million screening audiograms have been obtained on children at schools in Stockholm. The incidence of hearing loss in children of a particular age did not differ appreciably over the 14-year period analysed. In the age groups from 4 to 16, the incidence of temporary conductive hearing impairment decreased with age, whereas the incidence of permanent defects and particularly sensorineural high-frequency loss showed an increase with age.

For conductive hearing impairments there was no sex difference but for sensorineural defects there was a definite prepoderance of boys which was observed as early as 7 years of age.

The results of this general survey of the total material are supplemented and verified by the findings recorded in a more thorough examination at schools in Stockholm of all 13-year-old children born in 1948. The discussion of the results leads to the opinion that high-frequency loss in children would seem to be to a large extent of genetic origin.     

Ototoxic impact of cisplatin in pediatric oncology patients

OBJECTIVE: To describe hearing changes in a group of 28 children (age range, 8-180 mo) undergoing protocol-based cisplatin therapy. METHODS: Conventional, play audiometry, visual reinforcement audiometry (VRA), immittance audiometry, transient click evoked otoacoustic emissions (OAEs), and auditory brainstem response (ABR) evoked potentials were used to assess peripheral sensitivity and for threshold determination. RESULTS: Bilateral symmetrical high-frequency sensorineural hearing loss was noted in 9 of the 28 children (26%). Hearing loss was evident as early as 1 month after chemotherapy and as late as 50 months and was not dependent on individual or cumulative dosage of cisplatin. CONCLUSIONS: 1) Presence of sensorineural hearing loss was independent of individual and/or cumulative dosage of cisplatin; 2) audiologic assessment should be incorporated into a child's periodic medical evaluations after chemotherapy treatment, as onset of sensorineural hearing loss cannot be predicted; 3) personal hearing aids may be indicated for those children with hearing loss affecting the low- to mid-frequencies; a personal assistive listening device (frequency modulated system) may be more appropriate for losses above 3000 Hz; and 4) evaluation and intervention by a speech-language pathologist may be indicated to address possible articulation or language development problems consequent to hearing loss.     

Traumatic perilymphatic fistulas in children: etiology, diagnosis and management

Post-traumatic perilymphatic fistulas have been described following ear and temporal bone injury, particularly in the setting of temporal bone fractures. However, indications for exploratory surgery in cases of trauma without temporal bone fracture are vague and not well described. We describe three children who presented with symptoms suggestive of perilymphatic fistula (PLF) without an associated temporal bone fracture: two with penetrating tympanic membrane injuries and one with blunt temporal bone trauma. All had symptoms of hearing loss and vestibular disturbance. Two of the children cooperated with ear-specific audiologic assessment, which demonstrated sensorineural hearing loss (SNHL) on the traumatized side. The third child showed audiometric evidence of a SNHL on the injured side, but due to his age, the degree of severity of the SNHL was unable to be appropriately addressed prior to the patient being surgically managed. All three children underwent exploratory surgery and were found to have bony defects in the region of the oval window. All were repaired with fascial grafts to the oval and round windows with complete resolution of vestibular symptoms. However, two of the three patients with documented post-operative audiograms suffered from persistent SNHL on the injured side. We conclude that exploratory middle ear surgery is indicated in patients suffering from blunt or penetrating temporal bone or middle ear trauma who demonstrate persistent vestibular symptoms, sensorineural hearing loss or radiographic evidence of oval window pathology. As this is a limited number of patients, a larger series may be warranted to study the actual incidence of post-traumatic PLF in the child with persistent hearing loss and vertigo after head or ear trauma.     

Clinical predictors for hearing loss in children with bacterial meningitis

OBJECTIVES: To identify clinical risk factors that predict a higher incidence of hearing loss in children with bacterial meningitis, to determine the overall incidence of hearing loss in a large group of children proven by culture findings to have bacterial meningitis, and to compare clinical characteristics among patients with Streptococcus pneumoniae meningitis and Neisseria meningitidis meningitis. DESIGN: Retrospective review SETTING: Tertiary pediatric hospital. PATIENTS: A total of 171 children identified with bacterial meningitis who met inclusion criteria over a consecutive 10-year period. MAIN OUTCOME MEASURE: Presence of sensorineural hearing loss. RESULTS: Of 134 patients who underwent audiologic testing during their initial hospitalization, 41 (30.6%) were found to have at least a unilateral mild sensorineural hearing loss. The incidence of hearing loss was greater in patients with S pneumoniae meningitis than in patients with N meningitidis meningitis (35.9% and 23.9%, respectively). Length of hospitalization, development of seizures, elevated cerebrospinal fluid protein, and decreased cerebrospinal fluid glucose were significant predictors for hearing loss in children with bacterial meningitis. These factors were not found to be as strong a predictor for hearing loss in patients with N meningitidis meningitis. Stability of hearing was demonstrated with limited follow-up audiometry. CONCLUSIONS: Sensorineural hearing loss is a common sequela in children with bacterial meningitis. Identification of hearing loss in children with bacterial meningitis and early rehabilitation will lessen the long-term educational and social difficulties these children may experience.     

Fluctuating hearing loss and recurrent vertigo in otosclerosis

Summary This report summarizes audiologic and temporal bone findings in a 47-year-old white female with recurrent attacks of vertigo, tinnitus, and bilateral hearing loss. A series of audiograms, obtained over a 15-year period, showed a fluctuating mixed (sensorineural and conductive) hearing loss with a relatively flat configuration in the left ear and a relatively stable, mild sensorineural loss with a sloping contour in the right ear. Temporal bone studies revealed the existence of otosclerotic foci bilaterally. In the left ear, the otosclerotic focus had grown close to the utricular and lateral ampullary nerves, displaced and ankylosed the stapedial footplate, invaded the endosteal zone (1,000–2,000 Hz area), and deformed the lateral cochlear wall. In the right ear, the otosclerotic invasion was limited to the inferior portion on the promontory (vestibular cecum) and round window niche.Supported in part by NINCDS grant NS-10940, and E. C. McFadden Trust Research Fund     

Prognostic factors of profound idiopathic sudden sensorineural hearing loss

Profound idiopathic sudden sensorineural hearing loss is thought to have a poor prognosis, but few studies have focused on this condition. We aimed to assess the impact of patient factors, audiologic parameters, and salvage intratympanic steroid injection therapy on the prognosis of profound idiopathic sudden sensorineural hearing loss. The demographic, clinical, and audiologic data, degree of hearing recovery, and efficacy of intratympanic steroid injection therapy in 576 patients with profound idiopathic sudden sensorineural hearing loss (mean age 56.2 ± 14.9 years) who had been admitted at four tertiary referral centers between 2000 and 2011 were retrospectively reviewed. The mean hearing level at the initial presentation was 108.1 ± 9.5 dB. Many patients experienced vertigo (52.1 %) and tinnitus (77.4 %). At the 2-month follow-up, 172 (29.8 %) patients showed some degree of hearing recovery, but only 21 (3.6 %) patients recovered normal hearing. Further, the 116 patients who had received salvage intratympanic steroid injections showed a better audiologic outcome (improvement, 26.1 ± 24.3 vs. 15.7 ± 22.1 dB; P = 0.000) than those who had not (n = 429). In conclusion, a higher degree of hearing loss at the initial presentation indicates a poorer prognosis. Salvage intratympanic steroid injection therapy may improve the hearing of patients with profound idiopathic sudden sensorineural hearing loss after the failure of systemic steroid therapy.     

热休克蛋白70抗体检测对自身免疫性聋诊断的意义

目的 分析自身免疫性聋的听力学特点，探讨用免疫印迹法(Western blot)检测热休克蛋白70抗体在自身免疫性耳聋诊断中的意义。方法自身免疫性聋14例28耳，其它感音神经性聋8例16耳，正常对照6例12耳，采用免疫印迹法检测各组受试者血清中的热休克蛋白70抗体，并分别作临床听力学检查。结果自身免疫性耳聋者纯音听力图多数为平坦型，TEOAE和／或DPOAE未引出，听性脑干反应多数正常，轻、中度耳聋者可引出镫骨肌声发射，该组8例(57．1％)热休克蛋白70抗体阳性。其它感音神经性聋者纯音听力图多数为高频减退型和平坦型，TEOAE和DPOAE未引出，重度耳聋者ABR未引出，轻、中度耳聋者可引出镫骨肌声发射，1例(12．5%)热休克蛋白70抗体阳性。正常对照组1例(16．7％)热休克蛋白70抗体阳性。结论免疫印迹法检测热休克蛋白70抗体对自身免疫性耳聋诊断有参考价值，对该病的确诊还应结合临床听力学特点。     

Sudden hearing loss in intralabyrinthine haemorrhage in a child

This paper reports an unusual case in which aseptic meningitis presented with sudden sensorineural hearing loss (SSNHL) associated with intralabyrinthine haemorrhage (ILH). A seven-year-old girl presented with sudden right-sided hearing loss with dizziness. She did not have a previous history of bleeding disorders. This child was assessed using audiograms and magnetic resonance imaging (MRI). The patient's hearing loss was irreversible. Steroid therapy was not effective. SSNHL associated with ILH can be one of the negative prognostic factors in children.