Absence of sensorineural hearing loss in treated infants and children with congenital toxoplasmosis.

Educationally significant hearing loss has been reported in 10% to 15% of children with congenital toxoplasmosis. As part of a pilot study to assess feasibility and safety of prolonged therapy for congenital toxoplasmosis, 30 congenitally infected infants and children were evaluated for auditory function. Serial testing, beginning within 2 months of birth, was performed. Availability of auditory brainstem response (ABR) testing made evaluation at an earlier age than previously possible. Six (20%) of the 30 infants had mild to moderate conductive type hearing loss associated with otitis media. No infant or child had sensorineural hearing loss. The better outcome we observed compared to previous reports of a 15% to 26% incidence of sensorineural hearing loss and 10% to 15% incidence of educationally significant, bilateral hearing impairment may be related to early initiation and/or prolonged institution of antimicrobial therapy. Continued followup to exclude progressive hearing impairment and study of larger numbers of children are needed to verify these preliminary findings.     

Bullous myringitis with sensorineural hearing loss

Bullous myringitis is manifested by moderately severe otalgia associated with vesicles on the tympanic membrane and medial external auditory canal wall. This disease occurs mainly in young adults, most commonly in winter, and is usually preceded or accompanied by an upper respiratory infection. A reversible sensorineural loss developed in three of 22 patients. Complement fixation antibody studies performed on ten patients failed to implicate Mycoplasma pneumoniae, influenza virus types A or B, or adenovirus as the causal agent.     

Vestibular schwannoma in patients with sudden sensorineural hearing loss

Sudden sensorineural hearing loss (SSNHL) has several etiologies. It may be a presenting symptom of vestibular schwannoma (VS). This study aimed to establish the incidence of VS in patients with SSNHL, and we report several unusual cases among these patients. We reviewed retrospectively the charts and magnetic resonance imaging (MRI) findings of all adult patients who presented with SSNHL between 2002 and 2008. We utilized three-dimensional fast imaging with steady-state acquisition temporal MRI as a screening method. Of the 295 patients with SSNHL, VS was found in 12 (4%). All patients had intrameatal or small to medium-sized tumors. There were three cases with SSNHL in one ear and an incidental finding of intracanalicular VS in the contralateral ear. There were four cases of VS that showed good recovery from SSNHL with corticosteroid treatment. There were two cases that mimicked labyrinthitis with hearing loss and vertigo. A greater number of cases than expected of VS were detected in patients with SSNHL, as a result of increasing widespread use of MRI. Various unusual findings in these patients were identified. MRI would seem to be mandatory in all cases of SSNHL.     

Stapedectomy revision following sensorineural hearing loss

This study analyzes 71 stapedectomies that resulted in a sensorineural hearing loss, followed by a revision stapedectomy on the suspicion of an oval window fistula. The cases were divided between two primary stapedectomy techniques: a stainless steel Robinson prosthesis on a vein graft and a wire prosthesis with Gelfoam. The major differences between the surgical findings of the two groups were the fistula rate with the wire prosthesis was 10 times that with the Robinson prosthesis; the wire prosthesis was longer than necessary in 21% of the cases in which it was used; there was no finding of excess length with the Robinson prosthesis; and after revision stapedectomy, dizziness was lessened in 20% of the patients in the Robinson prosthesis group, in 60% of those in the wire prosthesis group, and in 75% of those with fistula. Surgical directions are given for revision stapedectomy following a sensorineural hearing loss.     

Unilateral sensorineural hearing loss rehabilitation.

The Audiant Bone Conductor has been heralded as an aid for use in conductive hearing loss; however, its possible use in unilateral sensorineural hearing loss (SNHL) has also been proposed. Between July 1987 and July 1989, profound unilateral sensorineural hearing loss has been rehabilitated in 43 patients using the Xomed Audiant Bone Conductor. Patients who were willing to participate in this clinical trial and who were felt to be good contralateral routing of signals (CROS) aid candidates were selected preoperatively. Audiometric followup, selection criteria, patient satisfaction, and complications are discussed.     

Incidence and factors associated with sensorineural and conductive hearing loss among survivors of congenital diaphragmatic hernia

Purpose

The reported incidence of sensorineural hearing loss (SNHL) in long-term survivors of congenital diaphragmatic hernia varies widely in the literature. Conductive hearing loss (CHL) is also known to occur in CDH patients, but has been less widely studied. We sought to characterize the incidence and risk factors associated with SNHL and CHL in a large cohort of CDH patients who underwent standardized treatment and follow-up at a single institution.

Methods

We retrospectively reviewed charts of all CDH patients in our pulmonary hypoplasia program from January 2004 through December 2012. Categorical variables were analyzed by Fisher’s exact test and continuous variables by Mann–Whitney t-test (p ≤ 0.05).

Results

A total of 112 patients met study inclusion criteria, with 3 (2.7%) patients diagnosed with SNHL and 38 (34.0%) diagnosed with CHL. SNHL was significantly associated with requirement for ECMO (p = 0.0130), prolonged course of hospitalization (p = 0.0011), duration of mechanical ventilation (p = 0.0046), requirement for tracheostomy (p = 0.0013), and duration of loop diuretic (p = 0.0005) and aminoglycoside therapy (p = 0.0003).

Conclusions

We have identified hearing anomalies in over 30% of long-term CDH survivors. These findings illustrate the need for routine serial audiologic evaluations throughout childhood for all survivors of CDH and stress the importance of targeted interventions to optimize long-term developmental outcomes pertaining to speech and language.     

Etiologic diagnosis of sensorineural hearing loss in adults

OBJECTIVE: To determine the etiology of adult-onset sensorineural hearing loss. STUDY DESIGN AND SETTING: This is a prospective cohort study of 60 adult subjects with bilateral sensorineural hearing loss of no obvious etiology by medical history and physical examination. These patients were evaluated at an academic medical center and underwent evaluation by high-resolution computed tomography of the temporal bone, autoimmune panel, and DNA testing for mutations of both the GJB2 gene and the mitochondrial DNA (1555A>G and 7445A>G). RESULTS: An etiologic diagnosis was achieved in 6 patients: cochlear otosclerosis, 1 case; dilated vestibular aqueduct, 1 case; a mitochondrial DNA 7445A>G mutation, 3 cases; and a mitochondrial DNA 1555A>G mutation, 1 case. CONCLUSION: This result underscores the importance of a search for the etiology of a hearing deficit in adult patients. There are specific interventions now available for the management of hearing-impaired patients with cochlear otosclerosis and mitochondrial DNA mutations.     

Idiopathic sudden sensorineural hearing loss in dialysis patients

Although sudden sensorineural hearing loss (SSNHL) affects chronic kidney disease (CKD) patients more frequently than non-CKD patients, few reports have described SSNHL in dialysis patients. We aimed to review the characteristics of SSNHL in chronic dialysis patients and evaluate treatment responses to steroid therapy. We retrospectively reviewed the records of dialysis patients diagnosed with idiopathic SSNHL at Asan Medical Center between January 2000 and December 2014. Pure tone and speech audiometry analyzes were performed before and 2?weeks and 2?months after treatment onset to evaluate outcomes. Twenty-two patients (11 men, 11 women; mean age: 49.9?±?11.7?years) were included; 16 (72%) and 6 (28%) had undergone hemodialysis and peritoneal dialysis, respectively, for a median of 49.2?±?41.4 (1–144) months. End-stage renal disease was most frequently caused by diabetic nephropathy (11 cases), chronic glomerulonephritis (1 case) and unknown factors (7 cases). Common accompanying symptoms included tinnitus (68.2%), ear fullness (45.5%) and vertigo (27.3%). The mean pure tone audiometry threshold at the initial presentation was 82.6?±?22.4?dB. At 2?months post-steroid treatment, 4 (18.2%), 4 (18.2%) and 6 (27.3%) patients exhibited a complete, partial, or slight recovery, respectively; 8 patients (36.3%) showed no improvement. Although we could not identify the specific cause of SSNHL in this population, our relatively large case series elucidates the precise clinical features of SSNHL in this population and demonstrates the outcomes of steroid treatment.     

Asymmetric sensorineural hearing loss in a community-based population

Asymmetric sensorineural hearing loss (ASNHL) is fairly common, but it can be an indication of retrocochlear pathology. The incidence of acoustic neuroma (AN) has been estimated at 1/100,000; however, the incidence of AN in patients with ASNHL is unknown. The limitation of health care resources challenges otolaryngologists to develop reasonable cost-containment guidelines for the evaluation of patients with ASNHL for the presence of retrocochlear pathology. A 5-year (1990 to 1994) retrospective study of all patients with ASNHL who were evaluated in a community-based general otolaryngology practice was performed. Demographic, historic, and audiologic data and results from ABRs and radiologic studies were summarized. ASNHL was present in 325 patients. Auditory brain stem response tests were performed in 179 patients (55%), and 92% (164 of 179) were normal. Patients with abnormal or inconclusive auditory brain stem responses and patients with severe SNHL were evaluated with radiologic studies (46 patients). Among the 193 patients who had diagnostic studies, 4 were found to have ANs, for a prevalence of 2.1%. The charge of diagnosis per AN was more than $41,000. In summary, a small percentage of patients with ASNHL have retrocochlear pathology, and the charge of diagnosis per AN can be excessive. A cost-containment approach for the evaluation and management of patients with ASNHL is proposed.     

Permanent sensorineural hearing loss following spinal anesthesia

A 25-year-old female developed permanent, fluctuating sensorineural hearing loss (SNHL), disabling vertigo, and tinnitus following an uneventful spinal anesthesia for cesarean section. At her first visit to the ear-nose-throat (ENT) department approximately 2 months postoperatively, pure-tone thresholds revealed profound SNHL on the right side whereas thresholds were within normal limits on the left side. The recruitment score (SISI) was 95% at 2000 Hz on the right side. Directional preponderance towards the right and the right canal paresis were evidenced by bithermal caloric testing. At follow ups the pure tone thresholds have shown some improvement, but fluctuating SNHL, disabling vertigo attacks, and tinnitus have remained. These findings imply a cochlear pathology causing endolymphatic hydrops possibly induced by lumbar puncture for spinal anesthesia.     

Bilateral versus unilateral sudden sensorineural hearing loss.

OBJECTIVES: To analyze the clinical characteristics and treatment results between bilateral (bi-) and unilateral (uni-) sudden sensorineural hearing loss (SSNHL). STUDY DESIGN AND SETTING: A retrospective study. METHODS: Three hundred twenty-four patients with SSNHL were classified into two groups; simultaneous bi-SSNHL (n = 16) and uni-SSNHL (n = 308). We compared clinical characteristics, medical history, hearing level, and treatment results between the 2 groups. RESULTS: The incidence of bi-SSNHL was 4.9 percent of overall patients with SSNHL. Bi-SSNHL occurs more commonly in patients of older age, with preexisting diabetes mellitus, and lipid panel abnormalities compared with uni-SSNHL. Ten patients (62.5%) in the bi-SSNHL group showed hearing recovery in 1 or both ears compared with 56.5 percent of patients with uni-SSNHL. Only 12 (37.5%) of all 32 ears recovered in bi-SSNHL, which was significantly lower than in uni-SSNHL. CONCLUSION: Bi-SSNHL has a very low incidence and lower recovery rate than uni-SSNHL. Recognition of similarities and differences between bilateral and unilateral SSNHL can help in counseling and managing the patients.     

Idiopathic sudden sensorineural hearing loss: a review

Idiopathic Sudden Sensorineural Hearing Loss (ISSHL) remains one of the major unsolved otologic emergencies. It is characterized by the onset of an unilateral sensorineural hearing loss developing within 24 hours, and averaging on pure tone audiogram at least 30 dB HL for three subsequent octave steps, with no marked vestibular symptoms and no identifiable cause. ISSHL is a syndrome covering several heterogeneous entities resulting from different pathogenetic mechanisms. At this time, the audiogram is the unique tool which may help clinicians to identify these entities and provide a classification based on 5 types of hearing loss. Numerous experimental and clinical studies have investigated the mechanisms by which infectious, ischemic, mechanic or immunologic insults may induce cochlear dysfunction. However, extrapolation to humans and rationale therapeutic approaches to ISSHL remain uncertain. SSHL being a diagnosis of exclusion, retrocochlear and neurologic etiologies should be eliminated. No argument allows to consider ISSHL a therapeutic emergency. More precisely, the experimental data presently available on cochlear physiology suggests that a treatment could have some chance to be effective if undertaken within minutes following the onset of ISSHL, a condition never encountered in daily practice. Conversely, it is not justifiable to impute the absence of hearing recovery to a delay in therapy. The various therapeutic strategies currently recommended are highly empirical and should be questionned in terms of cost-effectiveness, the most common being high-dose corticosteroids. New investigation tests are required for improving our approach to ISSHL.     

Bilateral sensorineural hearing loss complicating basal skull fracture

Otological damage is well-recognized following head injury. The commonest complication is hearing loss. We present a case of bilateral sensorineural hearing loss due to bilateral temporal bone fractures following an epileptic seizure, which is unique in the literature and illustrates the importance of this complication of head injury.     

Saccular damage in patients with idiopathic sudden sensorineural hearing loss without vertigo

OBJECTIVE: Saccule could be damaged in patients with idiopathic sudden sensorineural hearing loss (ISSHL) with vertigo and with high-frequency sensorineural hearing loss. Thus, the saccule might be deteriorated subclinically in ISSHL cases without vertigo. Therefore, we investigated saccular damage in ISSHL patients without vertigo through vestibular evoked myogenic potentials (VEMP). STUDY DESIGN: A prospective study. SUBJECTS AND METHODS: Fifty-two patients with ISSHL without vertigo were enrolled in the study. We identified VEMP in patients with ISSHL and analyzed the association of VEMP with initial hearing threshold, each threshold according to frequency, the type of audiogram, and hearing recovery. RESULTS: For cases with absent VEMP, we found significant differences between patients with 90 dB or more hearing loss and those with a hearing loss less than 55 dB with frequencies over 1000 Hz. Patients with profound hearing loss presented significantly high abnormal and absent VEMP than patients with audiograms of other types. CONCLUSION: These findings suggest that the subclinical deterioration of the saccular neuroepithelium is associated with patients with ISSHL having profound hearing loss at the high frequency.     

Efficacy of serologic testing in asymmetric sensorineural hearing loss.

PURPOSE: The goal of this study was to determine the efficacy of a detailed questionnaire, auditory brain stem response testing (ABR), MRI, and an extensive battery of serologic tests in diagnosing asymmetric sensorineural hearing loss (ASNHL). METHODS AND MATERIAL: Patients with audiograms demonstrating ASNHL of 10 dB or greater in 2 consecutive frequencies or 15 dB in any 1 frequency between 250 and 6000 Hz were asked to participate. Patients underwent MRI scanning of the cerebellopontine angle, internal auditory canals, and posterior fossa with gadolinium contrast, ABR, and an extensive battery of tests. The causative diagnosis was made by the individual clinician based on each patient's history, physical examination, and test results. RESULTS: Forty-five patients completed the study. A review of the data confirmed the utility of a detailed history and physical examination, MRI, and fluorescent treponemal antibody test in all cases. Erythrocyte sedimentation rate, glycosylated hemoglobin, Lyme antibody titers, and total hemolytic component (CH50) were helpful in selected cases. Thyroid function testing, complete blood count, Sequential Multiple Analysis-7, prothrombin time/partial thromboplastin time, lipid profile, and ABR were of no value in these patients. CONCLUSION: A careful history and physical examination, MRI, and fluorescent treponemal antibody test should be performed for the evaluation of all patients with ASNHL; however, more extensive serologic testing, including sedimentation rate, glycosylated hemoglobin, Lyme antibody titers, and CH50, should be selectively performed, based on a suggestive history or suspicious physical findings.     

Hearing preservation in patients with vestibular schwannomas with sudden sensorineural hearing loss.

OBJECTIVE: We evaluated hearing outcomes in patients with sudden hearing loss and vestibular schwannoma who underwent a hearing preservation operation for tumor resection in an effort to determine whether a history of sudden sensorineural hearing loss has an impact on subsequent hearing preservation surgery. METHODS: Retrospective chart review of 45 patients operated between 1990 and 1998. Patients were divided into "Recovery" (n = 22) and "No Recovery" (n = 23) groups based on preoperative hearing recovery. Hearing preservation was assessed using the AAO-HNS hearing classification system. RESULTS: Measurable hearing was preserved in 73% of patients, with 47% having good postoperative hearing (AAO-HNS Classes A-B). There was no significant difference in hearing outcome from patients presenting with progressive hearing loss (45% Classes A-B). There was also no difference in postoperative hearing between the "Recovery" and "No Recovery" groups. CONCLUSIONS: Patients with sudden hearing loss and vestibular schwannoma have the same chance of hearing preservation after tumor removal as those with progressive loss. Preoperative recovery of hearing is not predictive of hearing preservation. Available data support the nerve compression theory as the mechanism of sudden hearing loss in patients with vestibular schwannoma.