Auditory agnosia progressing to cortical deafness. Long-term follow up of a one-year-old child to age 37.

Background: There are two types of central auditory disorders due to pathology of the bilateral auditory cortices in adult patients: with residual hearing; with total hearing loss. However, long-term changes of hearing acuity over physical development time are unknown.

Objective: The aim of this case report was to illustrate the pathophysiology of auditory changes in a 1 year-3 months old child who was diagnosed with auditory agnosia as a sequel of herpes encephalitis and later developed cortical deafness during a 36-year follow-up.

Traits of Developmental Disorders in Adults With Listening Difficulties Without Diagnosis of Autism Spectrum Disorder And/or Attention-Deficit/Hyperactivity Disorder.

Some individuals have a normal audiogram but have listening difficulties (LiD). As many studies have investigated the relationship between listening and developmental disorders, the traits of developmental disorders might explain the symptoms of LiD. In this study, we examined the traits of developmental disorders of adults with LiD to help clarify the cause of LiD symptoms.

Temporal Bone Histopathology of Atypical Cogan Syndrome.

Cogan's syndrome is a rare disorder first clinically defined in 1945, characterized by nonsyphilitic interstitial keratitis and progressive audiovestibular symptoms. Later, patients with audiovestibular dysfunction and various types of inflammatory eye disease were classified as having atypical Cogan's syndrome. The etiology and pathogenesis of Cogan's syndrome remain largely unknown.

The error patterns of phonemes in children with prelingual hearing loss: A comparison between hearing aid and cochlear implant users.

Objective: To reveal differences in error pattern of phonemes and articulation between children using cochlear implants (CIs) and those using hearing aids (HAs) due to prelingual hearing disorder and help the education of children with prelingual hearing loss.

Method: Children with prelingual hearing loss who were receiving auditory-verbal preschool education at an auditory center for hearing-impaired children (Fujimidai Auditory Center, Tokyo, Japan) from 2010 to 2020 were analyzed retrospectively. All participants underwent pure tone audiometry and monosyllabic intelligibility tests.

Evoked Potentials by Tone Burst on the Auditory Cortices in Cats -Comparison of Off Responses in Awake and Anesthetized Conditions.

Background: Auditory Brainstem Response (ABR) recording in awake is essential to detect off-responses. This study clarified whether after-termination responses on ABR were offset responses, off-responses or a mixture of the two.

Methods: Evoked potentials in the auditory cortex of cats in response to tone burst stimuli were recorded, and off responses were examined with chronically implanted electrodes.

Auditory Attention Ability under Dichotic Dual-Task Situation in Adults with Listening Difficulties.

Introduction: Recent studies have reported poor cognition, such as attention and working memory, in adults with listening difficulties (LiD). However, they do not adequately describe the actual state of poor attention ability in adults with LiD. We examined the state of auditory attention in adults with and without LiD in tasks requiring multiple attention controls.

Cervical vestibular evoked myogenic potentials in 3-month-old infants: Comparative characteristics and feasibility for infant vestibular screening.

Objective: We compared the characteristics of air-conducted sound cervical vestibular evoked myogenic potential (ACS-cVEMP) and bone-conducted vibration cVEMP (BCV-cVEMP) among 3-month-old infants with normal hearing and sensorineural hearing loss (SNHL), and healthy adults to explore the feasibility and optimal strategies for infant vestibular screening.

Methods: 29 infants (58 ears) were divided into two groups according to hearing (group I: normal hearing ears; group II: SNHL ears), 20 healthy adults were defined as group III. The results of response rate, P13 and N23 latency, P13-N23 interval, amplitudes, and corrected interaural asymmetry ratio (IAR) were recorded and compared among three groups.

Comparison of vestibular ocular reflex and gross motor development in children with semicircular canal aplasia and hypoplasia.

Objective: To examine the effect of vestibular ocular reflex on gross motor development in children with semicircular canal aplasia and hypoplasia.

Study Design: Case series.

Materials And Methods: Children with congenital hearing loss underwent temporal bone computed tomography to assess their inner ear morphology.

Efficacy of transtympanic infusion of dexamethasone into the tympanic cavity in mice with acute sensorineural hearing loss associated with cytomegalovirus infection.

Background: There is no report on acute sensorineural hearing loss with congenital cytomegalovirus (cCMV) infection in basic experiments.

Aims/objectives: The aim of this study was to evaluate the effect of dexamethasone, an anti-inflammatory steroid, on acute sensorineural hearing loss in the mouse cytomegalovirus (MCMV) infection model mice.

Material And Methods: Sensorineural hearing loss model mice were divided into two groups, one with and one without intratympanic dexamethasone.

A High Risk of Missing Congenital Cytomegalovirus-Associated Hearing Loss through Newborn Hearing Screening in Japan.

It remains unclear to what extent newborn hearing screening (NHS) detects congenital cytomegalovirus (cCMV)-associated sensorineural hearing loss (SNHL) in Japan. This study aimed to clarify the NHS results and audiological characteristics of patients with cCMV-associated SNHL. A total of 541 individuals with unilateral or bilateral hearing loss of unknown etiology were examined for cCMV infection.

Patterns of hearing changes in women and men from denarians to nonagenarians.

Background: Hearing loss needs to be diagnosed and treated early, especially in older individuals, since presbycusis potentially increases the risk of depression and dementia. However, standard data on hearing thresholds across the life-span in Japanese individuals are lacking.

Methods: In a retrospective consecutive sample of 10681 native-Japanese speakers (37.

Investigation of the hearing levels of siblings affected by a single GJB2 variant: Possibility of genetic modifiers.

Objective: Variants in GJB2 can cause autosomal recessive deafness (DFNB1). There is evidence for genotype-phenotype correlations of GJB2 variants; however, several genotypes can cause varying levels of hearing loss likely attributable to differences in genetic or environmental background. As siblings share approximately 50% of their genetic background and usually have a common environmental background, analysis of phenotypes of siblings with a specific GJB2 variant may reveal factors relevant to phenotypic variation.

Assessment of speech perception in deaf or hard of hearing children who received auditory-verbal therapy with hearing aids or cochlear implants.

Objectives: To reframe the criteria for pediatric cochlear implants (CIs) in Japan, we investigated monosyllabic and word speech perception at the time of school entry in deaf or hard of hearing (D/HH) children who underwent auditory-verbal therapy (AVT) with hearing aids (HAs) or CIs in early childhood.

Methods: D/HH children who started AVT at Fujimidai Auditory Center for Hearing-Impaired Children before the age of 1 year and who underwent auditory assessment in the previous year of schooling (at the age of 5 or 6) from 2010 to 2020 were enrolled in this study. The results of hearing level tests with or without amplification and monosyllabic and word speech discrimination were assessed.

Charcot-Marie-Tooth Disease With Long-Term Follow-Up on Auditory Neuropathy-After Cochlear Implantation Or Hearing Aid Use.

Objective: To study the pathophysiology of Charcot-Marie-Tooth disease (CMT) with auditory neuropathy (AN) and to follow up cochlear implant or hearing aid use over the long term.

Study Design: Clinical capsule report.

Patients: Two adult CMT patients with AN.

Subcortical deafness as a subtype of auditory agnosia after injury of bilateral auditory radiations caused by two cerebrovascular accidents - normal auditory brainstem responses with I-VII waves and abolished consciousness of hearing.

Background: In central auditory disorders caused by damage of the cerebral hemispheres, there are cortical deafness and auditory agnosia. Although clinical cases of cortical deafness have been reported, little is known about the hearing problems and localized lesions associated with cortical deafness.

Aims/objectives: The aims of our research are to elucidate lesion sites associated with cortical deafness and to clarify why patients with cerebral lesions are not aware of any sound at all.

EABR measurements during cochlear implantation in one-year-old, infant, child, adult, and elderly patients.

Background: Clinical application of electrically-evoked intracochlear auditory brainstem responses (eABRs) for evaluation of brainstem maturity or aging changes has not been well investigated.

Aim/objective: We compare the eV latencies of intraoperative eABR measurements in one-year-olds, infants, children, adults, and the elderly, with the goal of investigating the changes in the brainstem auditory pathway due to development and aging.

Materials And Methods: We studied 58 ears of 51 patients who underwent cochlear implantation between 2013 and 2019 using MED-EL's Concerto or Synchrony implants with Flex28 or Flex soft electrodes.

Cochlear Implantation in Cases of Inner Ear Malformation: A Novel and Simple Grading, Intracochlear EABR, and Outcomes of Hearing.

Objective: To propose a simple grading of inner ear malformation (IEM) and investigate intracochlear electrical auditory brainstem response (EABR) and outcomes for hearing in terms of the novel grading system.

Study Design: Retrospective case review.

Setting: Tertiary referral center.

Differences in hearing levels between siblings with hearing loss caused by GJB2 mutations.

Objective: Hearing loss caused by GJB2 mutations is inherited in an autosomal recessive manner (DFNB1); thus siblings of an affected child have a 25% chance of also being affected. Hearing loss among subsequent siblings carrying the same GJB2 mutation is a concern for parents and a frequent topic of enquiry during genetic counseling. Evidence exists for genotype-phenotype correlations of GJB2 mutations; however, no analysis of differences in hearing among siblings, in whom the common genetic background may decrease variation, has been reported.

Vestibular Aging Process from 3D Physiological Imaging of the Membranous Labyrinth.

There is no three-dimensional (3D) technique to study the microanatomical structures of the in vivo 3D vestibular membranous labyrinth. Recent two MRI methods using a contrast agent can only depict the low-resolution imaging of endolymphatic hydrops. Therefore, we provide the new precise volume rendering algorithms to create the in vivo 3D vestibular membranous labyrinth images from high-resolution temporal bone low-dose CT data.

An unperceived acoustic stimulus decreases reaction time to visual information in a patient with cortical deafness.

Responding to multiple stimuli of different modalities has been shown to reduce reaction time (RT), yet many different processes can potentially contribute to multisensory response enhancement. To investigate the neural circuits involved in voluntary response initiation, an acoustic stimulus of varying intensities (80, 105, or 120 dB) was presented during a visual RT task to a patient with profound bilateral cortical deafness and an intact auditory brainstem response. Despite being unable to consciously perceive sound, RT was reliably shortened (~100 ms) on trials where the unperceived acoustic stimulus was presented, confirming the presence of multisensory response enhancement.

Electrically evoked ABR during cochlear implantation and postoperative development of speech and hearing abilities in infants with common cavity deformity as a type of inner ear malformation.

The electrically evoked auditory brainstem response (eABR) during cochlear implantation in common cavity (CC) deformity has not been clinically well studied. To investigate the eABR wave configuration during cochlear implantation, the postoperative development of hearing, and educational settings in infants with CC deformity. Nine infants who were congenitally deaf and found to have CC deformity and suspected cochlear nerve deficiency by temporal bone CT and MRI were studied.

Autosomal dominant optic atrophy with gene mutations accompanied by auditory neuropathy and other systemic complications in a Japanese cohort.

Purpose: This study aimed to describe the genetic and clinical characteristics of four Japanese patients with autosomal dominant optic atrophy (DOA) accompanied by auditory neuropathy and other systemic complications (i.e., DOA-plus disease).

A clinical and genetic study of 16 Japanese families with Waardenburg syndrome.

The purpose of this study is to profile the clinical and genetic features of Japanese Waardenburg syndrome (WS) patients and validate the W index. Sixteen Japanese WS families with congenital sensorineural hearing loss were included in the study. The inner canthal, interpupillary, and outer canthal distances (ICD, IPD, and OCD) were measured for all patients, and patients were screened for presence of PAX3, MITF, SOX10, and EDNRB mutations.