Functional connectivity changes in infants with varying degrees of unilateral hearing loss.

It has been proved that unilateral hearing loss (UHL) can cause functional connectivity alterations in adults. However, the mechanism of the human brain coping with the challenge of unilateral hearing deprivation at very early developmental phases remains poorly understood. Here, we performed a resting-state functional near-infrared spectroscopy (fNIRS) study on 3- to 10-month-old infants with varying degrees of unilateral hearing loss to investigate the effect of unilateral auditory deprivation in infants. Using network-based statistics, increased functional connectivity was observed in single-sided deafness (SSD) compared with normal hearing infants, and the right middle temporal gyrus was the most involved nodes. In addition, changes in cortical function in infants were related to the degree of hearing loss, with significantly increased functional connectivity in infants with severe to profound unilateral hearing loss compared with the ones with mild to moderate. Moreover, more significant cortical functional recombination changes were found in right-SSD than in left-SSD infants. For the first time, our study provides evidence for the effects of unilateral hearing deprivation on the early cortical development of the human brain, which would also act as a reference for intervention decisions in children with unilateral hearing loss in clinical settings.

Cochlear Nerve Deficiency in Pediatric Unilateral Hearing Loss and Asymmetric Hearing Loss.

INTRODUCTION: The rates of cochlear nerve abnormalities and cochlear malformations in pediatric unilateral hearing loss (UHL) are conflicting in the literature, with important implications on management. The aim of this study was to investigate the incidence of cochlear nerve deficiency (CND) in pediatric subjects with UHL or asymmetric hearing loss (AHL). METHODS: A retrospective chart review of pediatric subjects <18 years of age evaluated for UHL or AHL with fine-cut heavily T2-weighted magnetic resonance imaging (MRI) between January 2014 and October 2019 (n = 291) at a tertiary referral center was conducted. MRI brain and computed tomography temporal bone were reviewed for the presence of inner ear malformations and/or CND. Status of the ipsilateral cochlear nerve and inner ear was evaluated. Pure tone average (PTA) at 500, 1,000 and 2,000 Hz was assessed. RESULTS: 204 subjects with UHL and 87 subjects with AHL were included. CND (aplasia or hypoplasia) was demonstrated in 61 pediatric subjects with UHL (29.9%) and 10 with AHL (11.5%). Ipsilateral cochlear malformations were noted in 25 subjects with UHL (12.3%) and 11 with AHL (12.6%), and ipsilateral vestibular malformations in 23 (11.3%) and 12 (13.8%) ears, respectively. Median PTA was statistically significantly higher in ears with CND (98.33) than ears with normal nerves (90.84). DISCUSSION/CONCLUSION: Imaging demonstrated a high incidence of inner ear malformations, particularly CND, in pediatric subjects with UHL. Auditory findings indicated CND cannot be ruled out by thresholds alone as some CND ears did demonstrate measurable hearing. Radiologic evaluation by MRI should be performed in all patients within this population to guide counseling and management of hearing loss based on etiology, with implications on candidacy for cochlear implantation.

Isolated Incudostapedial Cholesteatomas: Unique Radiologic and Surgical Features.

OBJECTIVES: Congenital cholesteatomas originate from epithelial tissue present within the middle ear in patients with an intact tympanic membrane, no history of otologic surgery, otorrhea, or tympanic membrane perforation. They are diagnosed by a pearl-like lesion on otoscopy and computed tomography (CT) scan showing an expansile soft-tissue mass. We describe a series of patients with no prior otologic history presenting with progressive unilateral conductive hearing loss and normal otoscopy. The CT scans showed ossicular erosion without obvious soft-tissue mass. Surgery confirmed incudostapedial erosion found to be cholesteatoma. In this study, we characterize the clinical course of patients diagnosed with isolated incudostapedial cholesteatoma (IIC) and review possible pathologic mechanisms. METHODS: Retrospective review of IIC cases treated by the Department of Pediatric Otolaryngology, Rady Children's Hospital, San Diego, 2014 to 2020. Data included patient demographics, clinical features, imaging, surgical findings, and audiologic data. RESULTS: Five patients were diagnosed with IIC (3 [60%] female; mean age at presentation 10.7 years [range 5.5-16.0]). All patients presented with postlingual unilateral conductive hearing loss and normal otoscopy without any past otologic history; delay in diagnosis ranged from 4 months to several years. The CT scans showed ossicular chain erosion with an absent long process of the incus and/or stapes superstructure. All patients underwent middle ear exploration, revealing a thin layer of cholesteatoma in the incudostapedial region, confirmed by histopathology. Mean preoperative speech reception threshold was 55 dB and improved to a mean of 31 dB in the 4 patients who underwent ossicular chain reconstruction. CONCLUSION: Isolated incudostapedial cholesteatoma should be included as a possible etiology in pediatric patients with insidious onset of unilateral conductive hearing loss with normal otoscopy, unremarkable otologic history, and a CT scan showing ossicular abnormality/disruption without notable middle ear mass. These patients should be counseled preoperatively regarding the possibility of cholesteatoma and should undergo middle ear exploration with possible ossiculoplasty.

Evidence of a functional reorganization in the auditory dorsal stream following unilateral hearing loss.

Unilateral hearing loss (UHL) generates a disruption of binaural hearing mechanisms, which impairs sound localization and speech understanding in noisy environments. We conducted an original study using fMRI and psychoacoustic assessments to investigate the relationships between the extent of cortical reorganization across the auditory areas for UHL patients, the severity of unilateral hearing loss, and the deficit in binaural abilities. Twenty-eight volunteers (14 UHL patients) were recruited (twenty-two females and six males). The brain imaging analysis demonstrated that UHL induces a shift in aural dominance favoring the better ear, with a cortical reorganization located in the non-primary auditory areas, ipsilateral (same side) to the better ear. This reorganization is correlated not only to the hearing loss severity but also to spatial localization abilities. A regression analysis between brain activity and patient's performance clearly showed that the spatial hearing deficit was linked to a functional alteration of the posterior auditory areas known to process spatial hearing. Altogether, our study reveals that UHL alters the dorsal auditory stream, which is deleterious to spatial hearing.

Asymmetric sensorineural hearing loss and vestibular schwannoma: when to image?

PURPOSE OF REVIEW: We review the literature on the use of audiometric protocols in the guidance of when to obtain MRI for detection of vestibular schwannoma. This discussion will focus on the sensitivity, specificity, and cost-effective analysis of audiometric criteria of asymmetric sensorineural hearing loss (ASNHL) when used to decide when MRI scans should be performed. RECENT FINDINGS: The sensitivity for detecting vestibular schwannomas when invoking published audiometric protocols for triggering MRI acquisition in ASNHL ranged from 50 to 100%. Specificity of these protocols ranged from 23 to 83%. Such audiometric protocols are efficient, achieving sensitivity and specificity at these rates while reducing the screening rate to 18 to 35%. The reduced procurement of MRI while using such audiometric protocols is associated with annual cost savings of between 23 and 82%. While no definitive recommendations can be made from this review, some audiometric protocols offer a better balance of sensitivity and specificity than others. SUMMARY: Audiometric protocols for triggering MRI acquisition in ASNHL for evaluation of vestibular schwannoma can be both sensitive and specific. These are competitive measures, and so no protocol is both 100% sensitive and specific. Such protocols become less effective in populations with increased incidence of noise-induced hearing loss. Invocation of such audiometric protocols can considerably reduce the annual cost of MRI evaluation for vestibular schwannomas.

Auditory Comprehension in School-Aged Children With Normal Hearing and With Unilateral Hearing Loss.

Purpose The purpose of this study was to measure auditory comprehension performance in school-aged children with unilateral hearing loss (UHL) and with normal hearing (NH) in quiet and in the presence of child-produced two-talker babble (TTB). Method Listeners were school-aged children (7-12 years) with permanent UHL (n = 25) or NH (n = 14). Comprehension of three short stories taken from the Test of Narrative Language (Gillam & Pearson, 2004) was measured in quiet and in the presence of TTB at two signal-to-noise ratios (SNRs): (a) +6 dB and (b) the individualized SNR required to achieve 50% sentence understanding in the presence of the same TTB masker in a prior study (Griffin, Poissant, & Freyman, 2019). Target/masker spatial configuration was 0°/±60° azimuth. Results As a group, subjects with UHL demonstrated auditory comprehension abilities in favorable listening environments (i.e., quiet, +6 dB SNR) that were statistically equivalent to the NH group. However, in the most challenging listening condition (individualized SNR), many subjects with UHL demonstrated poorer comprehension performance than their age-matched peers with NH. Comprehension abilities were not associated with degree of UHL, unaided speech intelligibility index at 65 dB SPL in the impaired ear, side of UHL, or sex. Conclusions As a group, children with UHL demonstrated deficits in auditory comprehension compared to age-matched peers with NH in challenging listening environments. Findings highlight the importance of ensuring good SNRs for children with UHL.

The Role of Endolymphatic Hydrops in Patients with Pantonal Idiopathic Sudden Sensorineural Hearing Loss: A Cause or Secondary Reaction.

The purpose of this study was to investigate the presence of endolymphatic hydrops (EH) in both affected and unaffected ears of patients with pantonal unilateral idiopathic sudden sensorineural hearing loss (ISSNHL) using three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging (3D-FLAIR MRI) and further evaluate the significance of EH in this disorder. Twenty-seven ISSHL patients were enrolled in this study. 3D-FLAIR MRI was performed 24 h after intratympanic injection of gadolinium-diethylenetriaminepentaacetic acid (Gd-DPTA). The incidences of EH in the affected ears and contralateral unaffected ears were compared and the correlations of EH with vertigo or prognosis were analyzed using the Chi-square test. The results showed that the incidence of EH was 68.0% (17/25) in the affected ears and 34.8% (8/23) in the unaffected ears. There was a statistically significant difference between affected ears and unaffected ears in regard to the incidence of EH (P<0.05). There were no significant correlations of EH with vertigo (P=1.000) or with prognosis (P=0.359) in the affected ears. In conclusion, there is EH in the inner ear of patients with pantonal ISSNHL; EH is not related to vertigo, a concomitant symptom of ISSNHL, and the prognosis of this condition. The presence of EH may be a secondary reaction following the impairment of the inner ears with pantonal ISSNHL.

Topological reorganization after partial auditory deprivation-a structural connectivity study in single-sided deafness.

Growing evidence shows that partial auditory deprivation leads to extensive neural functional plasticity, which occurs not only in the auditory cortex but also in other sensory regions and cognitive areas. However, studies in structural topological properties are still limited, especially those investigating the relationship between structural connectome alterations and auditory abilities. To clarify this, we investigated white matter structural connectivity changes and the relationship between connection strength and hearing abilities in individuals with long-term single-sided deafness (SSD), a common form of partial hearing deprivation, using diffusion tensor imaging and network-based analysis. The results showed globally improved connection efficiency, locally weakened visual networks, and strengthened fronto-parietal sub-networks in SSD compared to normal hearing controls. Furthermore, a strong positive correlation between hearing abilities (including speech recognition in noise and sound localization) and connection strength, mainly in the fronto-parietal areas, was found in SSD. Our study reveals alteration of the structural network connections in SSD, especially in cognitive related networks, which showed close correlation with hearing abilities. Our findings provide new insights into topological white matter reorganization of the brain after partial sensory deprivation.

Assessment of the Clinical Benefit of Imaging in Children With Unilateral Sensorineural Hearing Loss: A Systematic Review and Meta-analysis.

IMPORTANCE: Imaging used to determine the cause of unilateral sensorineural hearing loss (USNHL) in children is often justified by the high likelihood of detecting abnormalities, which implies that these abnormalities are associated with hearing loss and that imaging has a positive contribution to patient outcome or well-being by providing information on the prognosis, hereditary factors, or cause of hearing loss. OBJECTIVES: To evaluate the diagnostic yield of computed tomography (CT) and magnetic resonance imaging (MRI) in children with isolated unexplained USNHL and investigate the clinical relevance of these findings. EVIDENCE REVIEW: Cochrane Library, Embase, PubMed, and Web of Science databases were searched for articles published from 1978 to 2017 on studies of children with USNHL who underwent CT and/or MRI of the temporal bone. Two authors (F.G.R. and E.N.B.P.) independently extracted information on population characteristics, imaging modality, and the prevalence of abnormalities and assessed the studies for risk of bias. Eligibility criteria included studies with 20 or more patients with USNHL who had CT and/or MRI scans, a population younger than 18 years, and those published in English. MAIN OUTCOMES AND MEASURES: The pooled prevalence with 95% CI of inner ear abnormalities grouped according to finding and imaging modality. FINDINGS: Of 1562 studies, 18 were included with a total of 1504 participants included in the analysis. Fifteen studies were consecutive case studies and 3 were retrospective cohort studies. The pooled diagnostic yield for pathophysiologic relevant findings in patients with unexplained USNHL was 37% for CT (95% CI, 25%-48%) and 35% for MRI (95% CI, 22%-49%). Cochleovestibular abnormalities were found with a pooled frequency of 19% for CT (95% CI, 14%-25%) and 16% for MRI (95% CI, 7%-25%). Cochlear nerve deficiency and associated cochlear aperture stenosis had a pooled frequency of 16% for MRI (95% CI, 3%-29%) and 44% for CT (95% CI, 36%-53%), respectively. Enlarged vestibular aqueduct (EVA) was detected with a pooled frequency of 7% for CT and 12% for MRI in children with USNHL. CONCLUSIONS AND RELEVANCE: Imaging provided insight into the cause of hearing loss in a pooled frequency of about 35% to 37% in children with isolated unexplained USNHL. However, none of these findings had therapeutic consequences, and imaging provided information on prognosis and hereditary factors only in a small proportion of children, namely those with EVA. Thus, there is currently no convincing evidence supporting a strong recommendation for imaging in children who present with USNHL. The advantages of imaging should be carefully balanced against the drawbacks during shared decision making.

Unilateral hearing loss in a 94-year-old patient.

A case report of a 94-year-old, previously well male patient who presented with fever thought to be caused by community acquired pneumonia, new unilateral hearing loss and reduced consciousness. Despite antibiotic treatment he continued to deteriorate. Brain imaging with computer tomography and magnetic resonance imaging revealed a left otomastoiditis with osteomyelitis of the skull base, associated with an adjacent subdural empyema. He was also found to have a venous sinus thrombosis, most likely secondary to otitis media. He was managed with intravenous antibiotics, anticoagulation, grommet insertion and a hearing aid and he made a good recovery. This case reminds us to consider otitis media in older patients who present with hearing loss and fever. Otitis media can lead to serious complications including subdural empyema and osteomyelitis of the skull base.

Vestibular and balance function is often impaired in children with profound unilateral sensorineural hearing loss.

RATIONALE: Children with unilateral deafness could have concurrent vestibular dysfunction which would be associated with balance deficits and potentially impair overall development. The prevalence of vestibular and balance deficits remains to be defined in these children. METHODS: Twenty children with unilateral deafness underwent comprehensive vestibular and balance evaluation. RESULTS: Retrospective review revealed that more than half of the cohort demonstrated some abnormality of the vestibular end organs (otoliths and horizontal canal), with the prevalence of end organ specific dysfunction ranging from 17 to 48% depending on organ tested and method used. In most children, impairment occurred only on the deaf side. Children with unilateral deafness also displayed significantly poorer balance function than their normal hearing peers. CONCLUSIONS: The prevalence of vestibular dysfunction in children with unilateral deafness is high and similar to that of children with bilateral deafness. Vestibular and balance evaluation should be routine and the functional impact of combined vestibulo-cochlear sensory deficits considered.

MR evaluation of encephalic leukoaraiosis in sudden sensorineural hearing loss (SSNHL) patients.

Epidemiological evidence suggests a strict correlation between sudden sensorineural hearing loss (SSNHL) and cerebrovascular disorders. Leukoaraiosis represents a diffuse alteration of the periventricular and subcortical white matter. The aim of our study was to verify if the presence of white matter hyperintensity (WMH) was higher in patients affected by SSNHL compared to controls and evaluate the correlation between WMH and the cardiovascular risk factors, hearing level, and the response to therapy in SSNHL patients. The study group included 36 subjects affected by unilateral SSNHL. Thirty-six age- and sex-matched normal subjects with a negative history of SSNHL were used as controls. All patients underwent magnetic resonance imaging (MRI) (1.5 Tesla GE Signa) and the extent of leukoaraiosis was assessed with the Fazekas scale. The results of the present study demonstrate a high prevalence of WMH in SSNHL patients compared to controls confirming the hypothesis of a vascular impairment in SSNHL patients. The higher recovery rate in patients with greater periventricular white matter hyperintensity (PWMH) may suggest a vascular etiology that is still responsive to medical treatment. We aim to expand both the number of patients and the controls to avoid the limitation of the still small number to warrant solid scientific conclusions.

Evaluating diagnostic yield of computed tomography (CT) and magnetic resonance imaging (MRI) in pediatric unilateral sensorineural hearing loss.

INTRODUCTION: Options for imaging for evaluation of pediatric patients with unilateral sensorineural hearing loss (USNHL) include computed tomography (CT) and magnetic resonance imaging (MRI). Although both CT and MR imaging provide valuable information in the evaluation of pediatric patients with USNHL, debate remains regarding which imaging modality is most ideal and should be the preferred study for these children. The objective of this study is to evaluate and compare the diagnostic yield of CT versus MRI in children with USNHL. METHODS: A multi-institutional retrospective chart review was conducted. Pediatric patients with hearing loss (diagnosis codes 389.00-389.22) seen between 2010 and 2012 at three tertiary care centers were identified. Only patients with USNHL and imaging studies were reviewed and results of CT and MRI for each patient were examined and compared. Cochleovestibular or central nervous system findings known to directly correlate to SNHL were noted as positive findings on imaging. McNemar's test was used to compare patients with positive CT and MRI results. RESULTS: A total of 219 patients between the ages of 0-18 years with USNHL who underwent CT and/or MRI were identified. Imaging abnormalities were found in 41/96 patients who underwent MRI with overall diagnostic yield of 42.7% and 69 of 188 patients who underwent CT with overall diagnostic yield of 36.7%. For patients who underwent both imaging modalities (n = 65), there was no statistically significant difference in positive findings detected by CT vs MRI (p > 0.05). CONCLUSIONS: Both CT and MR imaging have similar overall diagnostic yield when used to evaluate children with USNHL. Parents and patients should be counseled regarding cost, test duration, radiation exposure, need for sedation, and diagnostic accuracy associated with each imaging modality and these factors should be considered to select the appropriate diagnostic study.

Extremely common radiographic finding of cochlear nerve deficiency among infants with prelingual single-sided deafness and its clinical implications.

OBJECTIVES: To clarify the common radiographic findings of audiologically documented prelingual single-sided deafness (SSD) and identify the prevalence of cochlear nerve deficiency (CND) in SSD infants referred from the newborn hearing screening program. METHODS: Between March 2012 and March 2017, the records of all infants referred to our otology clinic after undergoing newborn hearing screening program were retrospectively reviewed. Twenty-four consecutive well infants without risk factors who had a confirmed diagnosis of prelingual SSD under the age of 1 year and who underwent internal auditory canal (IAC) magnetic resonance imaging (MRI) were included. The sizes of cochlear nerve (CN), IAC, and cochlear nerve canal (CNC) were measured on MRI. The presence of CND was visually determined by comparing the CN size to the ipsilateral facial nerve (FN) in the affected side via an oblique sagittal view of IAC MRI and defined when CN was absent or smaller than FN. RESULTS: CND was seen in all 24 deaf ears (100%) on MRI. There was one with incomplete partition type I, and another with combined cochleovestibular nerve absence. Twenty-four subjects demonstrated either an absent (20/24, 83.3%) or small (4/24, 16.7%) CN. When the absent and small CN groups were compared, the former group had a higher prevalence of narrow CNC and narrow IAC. Of the 20 infants without identifiable CN on the affected side, 17 (85%) had narrow IAC and 17 (85%) had narrow CNC. In the 20 ears with absent CN, only one had both normal-sized IAC and CNC. CONCLUSION: The contribution of CND to prelingual SSD in Korean infants reached 100%, according to IAC MRI alone.

Duplicated internal auditory canal with inner ear malformation: Case report and literature review.

Internal auditory canal anomalies are rare. Narrow internal auditory canal is believed to occur as a result of aplasia or hypoplasia of the vestibulocochlear nerve. Narrow duplication of the internal auditory canal is considered to be very rare. Narrow duplication of the internal auditory canal with inner ear malformation has been reported in only 3 cases. We present 2 cases of narrow duplication of the internal auditory canal with inner ear malformation. The first case had inner ear malformation on only one side and the second case had inner ear malformation on both sides. The embryogenesis may be different between internal auditory canal and inner ear.

Unilateral Sensorineural Hearing Loss: Medical Context and Etiology.

OBJECTIVE: Unilateral sensorineural hearing loss (USNHL) is known to impact on school performance and social skills during childhood, but the etiologies remain unclear. The aim of this study was to assess various etiologies and to study the clinical contexts in this population. METHODS: The study is a retrospective review. Characteristics of hearing loss (HL), audiometric parameters, imaging, and genetic and medical contexts were analyzed. RESULTS: Eighty children were included. USNHL was profound in 68%, could be progressive in 19%, and become bilateral in 7.5% of cases. Inner ear malformations were identified in 41% of cases; cochlear nerve deficiency (CND) was frequent (33%). Cytomegalovirus (CMV) infection and genetic syndromes were confirmed in 10 and 6% of cases, respectively. CONCLUSION: Long-term hearing follow-up remains useful in USNHL as it can become bilateral. Looking to etiology, MRI should be the gold standard, as CND is frequently observed and screening for CMV infection should be systematic. Genetic etiologies appear to be different compared to bilateral HL. Further genetic research in this domain is needed.

Concomitant imaging and genetic findings in children with unilateral sensorineural hearing loss.

OBJECTIVE: To describe the concomitant imaging and genetic findings in children diagnosed with non-syndromic unilateral sensorineural hearing loss. METHODS: A retrospective cohort study was conducted of 60 children diagnosed between January 2005 and December 2015 in a tertiary-level paediatric institution. RESULTS: Average age at diagnosis was 4.3 years. All children were considered non-syndromic. Hearing loss was categorised as mild (17 children), moderate (17 children), severe (7 children) or profound (19 children). Imaging was performed in 43 children (71.66 per cent). Nineteen patients (44.2 per cent) had positive computed tomography or magnetic resonance imaging findings. Genetic testing was performed in 51 children (85 per cent). Sixteen children (31 per cent) tested positive to connexin 26 (GJB2); 1 patient (2 per cent) had a homozygous mutation of GJB2 and 15 were heterozygous carriers. Amongst children who tested positive as heterozygous carriers of a GJB2 mutation, there was a high rate of positive imaging findings (47 per cent compared to 37.2 per cent in the total cohort). A genetic abnormality was confirmed in 50 per cent of children with positive imaging findings who underwent genetic testing. CONCLUSION: Rates of concomitant imaging and genetic findings suggest that both investigations are of value in the study of these patients.

Etiology of single-sided deafness and asymmetrical hearing loss.

CONCLUSIONS: The present study revealed that various etiologies are involved in single-sided deafness (SSD), and that the cause of SSD and asymmetrical hearing loss (AHL) differed greatly between congenital/early-onset cases and adult cases. Clarification of the etiology is the first step toward providing appropriate intervention. OBJECTIVES: The study aimed to clarify the etiology of SSD and AHL patients. METHODS: The etiology of a total of 527 SSD or AHL patients who visited Shinshu University Hospital between 2006 and 2016 were analyzed by imaging as well as serological tests for mumps virus, and CMV DNA testing. RESULTS: In our cohort of congenital/early-onset SSD (n = 210), the most prevalent cause in children was cochlear nerve deficiency (43.7%; 87 of 199 patients undergoing CT and/or MRI), followed by CMV infection, mumps infection, anomalies of the inner ear, ANSD, and other rare etiologies. In contrast, half of the adult SSD patients presented with idiopathic sensorineural hearing loss, followed by various types of otitis media, cerebellopontine angle tumor and other rare etiologies.

High-Resolution fMRI of Auditory Cortical Map Changes in Unilateral Hearing Loss and Tinnitus.

Animal models of hearing loss and tinnitus observe pathological neural activity in the tonotopic frequency maps of the primary auditory cortex. Here, we applied ultra high-field fMRI at 7 T to test whether human patients with unilateral hearing loss and tinnitus also show altered functional activity in the primary auditory cortex. The high spatial resolution afforded by 7 T imaging allowed tonotopic mapping of primary auditory cortex on an individual subject basis. Eleven patients with unilateral hearing loss and tinnitus were compared to normal-hearing controls. Patients showed an over-representation and hyperactivity in a region of the cortical map corresponding to low frequencies sounds, irrespective of the hearing loss and tinnitus range, which in most cases affected higher frequencies. This finding of hyperactivity in low frequency map regions, irrespective of hearing loss range, is consistent with some previous studies in animal models and corroborates a previous study of human tinnitus. Thus these findings contribute to accumulating evidence that gross cortical tonotopic map reorganization is not a causal factor of tinnitus.