The Management of Pediatric Otitic Hydrocephalus: Case Report.

Otitic Hydrocephalus (OH) is one of the most significant life-threatening complications of otological infections. Given their low prevalence and non-specific ear symptoms, this complication requires a high index of suspicion for diagnosis. In this case report, we aim to provide an analysis of OH and describe common clinical signs and symptoms, treatment options, morbidity and mortality.

Long term results of glass ionomer ossiculoplasty.

OBJECTIVE: To analyze and compare the early and late post-operative results of glass ionomer bone cement (GIBC) used in ossiculoplasty. METHODS: The pre-operative, early post-operative (3 months) and late post-operative (> 2 years) audiometric findings, namely the pure-tone average (PTA), bone conduction threshold (BCT) and air-bone gap (ABG) of 40 patients who underwent GIBC ossiculoplasty for different etiologies were analyzed. Early and late results were compared. Also, the patients were grouped in terms of prognostic factors and applied ossiculoplasty techniques, and the results were compared between the groups. RESULTS: There were statistically significant improvements in the pure-tone average and air-bone gap of the patients in the early post-operative period (PTA from 59.60 ± 15.95 to 40.37 ± 17.83 and ABG from 37.12 ± 11.18 to 19.78 ± 10.41, p < 0.001 for both). There were no statistically significant changes in any of the audiometric parameters in the late post-operative period (PTA from 40.37 ± 17.83 to 39.79 ± 17.91, ABG from 19.78 ± 10.41 to 19.32 ± 9.60, BCT from 17.99 ± 12.71 to 18.31 ± 13.99, p > 0.05 for all). Presence of tympanosclerosis was found to be the only prognostic factor to affect the outcome. CONCLUSION: GIBC is a safe and reliable material for ossiculoplasty, which maintains its ability to conduct sound in the long-term follow-up.

Unimodal versus bimodal auditory stimulation in inner ear malformations: Cognitive, language, and motor performance.

Purpose: New perspectives on rehabilitation options for inner ear malformations have still been studied in the literature. This study investigated the cognitive, language, and motor skills of auditory brainstem implant (ABI) users in unimodal and bimodal groups. Methods: The motor competency of the participants was assessed with Bruininks-Oseretsky Motor Proficiency Test-2 Short Form (BOT2 SF). Language performance was evaluated by the test of Early Language Development-3 and Speech Intelligibility Rating. Word identification, sentence recognition tests, and Categories of Auditory Performance were used to assess auditory perception skills. To examine the cognitive performance, Cancellation Test and Gesell Copy Form were administered. All the tests were conducted in a quiet environment without any distractions. Results: The participants were divided into two groups: (1) 17 children in the unimodal group and (2) 11 children in the bimodal (who used a cochlear implant on one side and ABI on the other side) group. There were significant correlations between the chronological age of participants and BOT2 SF total score, cancellation tests, auditory perception tests, and language performance. Similarly, there were significant correlations between the duration of ABI use and auditory perception tests, language performance, cancellation test, and some BOT2 SF subtests (r = -0.47 to -0.60, p < .001). There was no significant difference between the unimodal and bimodal groups in any task (p > .05). However, there were moderate-to-strong correlations among the auditory perception tests, cancellation test, language test, and BOT2 SF total score and subtests (r = 0.40 to 0.55, p < .05). Conclusion: Although there were no significant differences between bimodal and unimodal groups, a holistic approach, which indicates that hearing and balance issues can have broader impacts on a person's physical, emotional, social, and psychological aspects, should be used in the assessment process. Level of Evidence: Level 4.

Speech recognition and quality of life outcomes of adults with cochlear implants following a quarter-century of deafness: what should be the maximum duration?

PURPOSE: This study was aimed at examining the pre- and post-cochlear implant (CI) speech recognition and quality of life results of postlingually deaf adult CI users with a duration of deafness (DoD) > 25 years and determining the maximum DoD limit. METHODS: We enrolled 54 postlingually deaf CI users and divided them into ages ≤ 60 and > 60 years and DoDs ≤ 25 and > 25 years. All participants were evaluated using multisensory measures (auditory and auditory + visual) and open-set Speech Recognition Test (SRT) before CI and 3 years postoperatively. They were administered with The Hearing Handicap Inventory for the Elderly (HHIE) to determine the effects of hearing impairment on daily life. RESULTS: DoD and open-set SRT for auditory and auditory + visual stimuli showed a strong negative linear relationship (r = - 0.506, p < 0.01). Open-set SRT scores of patients with DoD aged ≤ 25 and > 25 years (p < 0.01) differed significantly. The chronological age and HHIE scores in social and emotional subfactors showed a strong negative linear relationship (r = - 0.519, p < 0.01). CONCLUSIONS: The present study showed that the number of years was a major factor determining that postlingual adults with profound hearing loss had hearing loss. The results support CI use as soon as possible in adults to prevent degeneration of the auditory pathways and possible central remodeling. However, auditory rehabilitation outcomes in adults using CI vary widely. Investigating the causes of this variability contributes to audiology.

Flocculus Herniation into the Internal Acoustic Canal in Incomplete Partition Type I: A Case Report.

In this study, we present the first case with cerebellar herniation into the internal acoustic canal in incomplete partition type I anomaly. Cerebellar herniation into the internal acoustic canal is very rare with only a few cases reported in the literature. Although it is a rare clinical situation, cerebellar herniation into the internal acoustic canal may be seen in patients with incomplete partition type I. We presented magnetic resonance imaging findings of a 3-year-old girl with a history of meningitis, middle ear effusion, and bilateral congenital sensorineural hearing loss. Magnetic resonance imaging showed bilateral incomplete partition type I malformation and an additional flocculus herniation into the right internal acoustic canal. In the presented case, predisposition to cerebrospinal fluid leak in incomplete partition type I anomaly may be the reason for cerebellar herniation into internal acoustic canal. Also, possible increased intracranial pressure due to meningitis may be a contributing factor.

Hearing Loss Related to Gene Mutations in Distal Renal Tubular Acidosis.

INTRODUCTION: Distal renal tubular acidosis (dRTA) is a disease that may develop either primarily or secondarily, resulting from urinary acidification defects in distal tubules. Hearing loss may accompany primary forms of dRTA. This study aims to determine the characteristics of hearing loss due to different gene mutations in patients with dRTA. METHODS: Behavioral and electrophysiological audiological evaluations were performed after otolaryngology examination in 21 patients with clinically diagnosed dRTA. Radiological imaging of the inner ear (n = 9) was conducted and results of genetic analyses using next-generation sequencing method (n = 16) were included. RESULTS: Twenty-one patients with dRTA from 20 unrelated families, aged between 8 months and 33 years (median = 12, interquartile range = 20), participated. All patients with ATP6V1B1 mutations (n = 9) had different degrees of hearing loss. There was one patient with hearing loss in patients with ATP6V0A4 mutations (n = 6). One patient with the WDR72 mutation had normal hearing. Large vestibular aqueduct syndrome (LVAS) was detected in 6 (67%) of 9 patients whose radiological evaluation results were available. CONCLUSIONS: LVAS is common in patients with dRTA and may influence the type and severity of hearing loss in these patients. The possibility of both congenital and late-onset and progressive hearing loss should be considered in dRTA patients. A regular audiological follow-up is essential for the early detection of a possible late-onset or progressive hearing loss in these patients.

Speech Perception and Sound Localization Skills in Inner Ear Malformations: Children With Incomplete Partition Type-II.

OBJECTIVE: The present study aimed to evaluate binaural auditory skills in bimodal and bilateral pediatric cochlear implant (CI) users with incomplete partition type-II (IP-II) and to reveal the effect of IP-II on performance by comparing the results to pediatric CI users with normal cochlear morphology. STUDY DESIGN: Cross-sectional study. SETTING: Tertiary referral center. METHODS: Forty-one CI users (mean age 8.8 ± 1.9) were grouped as bimodal (BIM-IP) and bilateral (BIL-IP) users with IP-II; bimodal (BIM-N) and bilateral (BIL-N) users with normal cochlear anatomy. Speech perception in noise and sound localization skills were compared under 2 conditions; binaural (bilateral or bimodal) and monaural (first CI alone). RESULTS: BIM-IP and BIL-IP showed no performance difference in binaural tasks. The BIM-N group showed remarkably poor performance in comparison to the groups of BIL-IP (p = .007), BIM-IP (p < .001), and BIL-N (p = .004) in terms of speech-in-noise skills. In sound localization abilities, similar significant differences were found between the group of BIM-N and the groups of BIL-IP (p = .001), BIM-IP (p < .001), and BIL-N (p = .004). All groups showed statistically significant improvements in binaural condition on both tasks (p < .05). CONCLUSION: We revealed that bilateral and bimodal pediatric CI users with IP-II benefitted from implantation as much as bilateral users with normal anatomy. Differences in residual hearing between groups may explain the poor performance of bimodal users with normal cochlear morphology. To the best of our knowledge, it is the first study to unveil binaural performance characteristics in children diagnosed with a specific inner ear malformation subgroup.

Evaluation of the non-auditory responses in individuals with auditory brainstem implant.

OBJECTIVES: After auditory brainstem implant (ABI) surgery, stimulation of certain cranial nerves may result in a non-auditory response, and the electrodes that stimulate these nerves may be deactivated. The goals of this study are to compare the number of active electrodes in the initial activation and the last fitting, to investigate non-auditory response types and their frequency as a result of non-auditory stimulation, to compare the placements of deactivated electrodes as a result of non-auditory stimulation in the initial activation and the last fitting. METHODS: The computer software system was used to perform a retrospective analysis of the fitting data of 69 ABI users who underwent auditory brainstem implant surgery between January 1997 and January 2019. The non-auditory response types, deactive electrodes, and the positioning of the deactive electrodes horizontally and vertically were recorded in these users during the initial activation and the last fitting. RESULTS: There was no statistically significant difference between the number of active electrodes in the initial activation and the last fitting. The proportion of the users with deactive electrodes in the initial activation and the last fitting was not statistically significant different. In the horizontal and vertical placement classification, the placement of the deactive electrodes was not statistically different between initial activation and last fitting. The most common type of non-auditory response was facial nerve stimulation at the initial activation and no auditory perception at the last fitting. According to the difference between the number of active and deactive electrodes in the initial activation and the last fitting, as well as the auditory and non-auditory responses, it was found that the ABI users were statistically different between the initial activation and the last fitting. CONCLUSION: The results of this study show that not only auditory but also non-auditory responses occur in most ABI users. In addition, to the best of our knowledge, this study is the first to examine the frequencies of non-auditory response types, and the placement of the electrodes that cause these responses according to horizontal and vertical classifications.

Written language skills in children with auditory brainstem implants.

PURPOSE: This study aimed to assess the written language skills of children with auditory brainstem implants (ABI). METHODS: In this study, 15 children (from second to eighth grades) with ABI were evaluated for their written language abilities using a written expression skill assessment form. Five different features of written expression points were scored and analyzed, yielding a composite score for written expression skills. RESULTS: This study showed that all children with ABI needed more verbal cues than spontaneously written samples. Moreover, these children used short and simple sentences with limited vocabulary and repeated words and sentences. Furthermore, these children were deficient in writing an introduction, the body, and the conclusion paragraphs and could not write events in a logical sequence. CONCLUSIONS: The written language skills of children with ABI depend on age at implantation, duration of implant use, and additional handicaps. Written expression skills in children with ABI are highly complex skills. The findings highlight the importance of ABI during the critical language development period and the enhancement of training programs for written language skills in children who underwent ABI.

X-linked deafness/incomplete partition type 3: Radiological evaluation of temporal bone and intracranial findings.

PURPOSE: X-linked deafness (XLD) is a rare disease, characterized by typical cochlear incomplete partition type 3 anomaly (IP-III). Accompanying hypothalamic anomalies were also recently described. The purpose of this study was to document the temporal bone and intracranial imaging findings in a series of patients with XLD with a review of the literature, to better understand this anomaly. METHODS: The CT and MRI studied of 13 XLD patients were retrospectively evaluated. All structures of the otic capsule (OC) were subjectively and retrospectively assessed. The OC thickness and the size of the cochlea were measured and compared to the age-matched control group. Intracranial structures were also evaluated with specific attention to the hypothalamic region. RESULTS: All cases had bilateral IP-III anomaly, bulbous internal auditory canals (IACs), absent bony modiolus with preserved interscalar septa, intact cochleovestibular, and facial nerves. OC thickness was decreased in all cases compared to the control group (p<0.001). In XLD patients, the cochlea had decreased transverse dimension and increased height compared to the control group (p< 0.001). Five patients (38.4%) had bilateral cystic structures adjacent to the vestibule and/or semicircular canals (SCCs). Hypothalamus was thickened or had a lobular appearance in all cases (subtle in one). Additionally, hamartoma-like appearance of the hypothalamus was present in half. CONCLUSION: XLD is a rare inner ear anomaly that is frequently associated with hypothalamic malformations. The OC thickness of IP-III patients appears to be decreased with accompanying decreased transverse dimension of the cochlea which could have implications in electrode selection during cochlear implantation. Cystic /diverticular lesions surrounding the vestibule and semicircular canals are also frequently seen but a rarely reported finding.

Revision Surgery for Pediatric Nontumor Auditory Brainstem Implant Users: Audiological, Rehabilitative, and Surgical Outcomes.

OBJECTIVE: To report the audiological, rehabilitative, and surgical outcomes of revision surgery for pediatric auditory brainstem implant (ABI) users. STUDY DESIGN: Retrospective cohort. SETTING: Tertiary referral center. PATIENTS: Five pediatric ABI users who had revision surgery for device malfunctions. INTERVENTIONS: Revision surgery for ABI malfunctions. MAIN OUTCOME MEASURES: The findings of free-field audiometry with the device, the Meaningful Auditory Integration Scale, and the pattern discrimination, word identification, sentence recognition, and expressive and receptive language tests before the device failure and after revision surgery were obtained from the patient records and compared. RESULTS: The revision rate for pediatric ABI was 6.45%. The Meaningful Auditory Integration Scale and expressive-receptive language scores showed improvements following revision surgery, while the aided thresholds, pattern perception, and word identification scores did not change. Individual differences in performance for these measures were observed. CONCLUSION: Equal or improved performance after the revision surgeries in the current study showed that revision surgery is successful and important for pediatric ABI users. It is essential to consider remedying the loss of auditory input in sensitive periods of pediatric development.

Factors affecting phoneme discrimination in children with sequential bilateral cochlear implants.

OBJECTIVES: To investigate the effects of a number of variables on phoneme discrimination (PD) performance in children with sequential bilateral cochlear implants (SeqBiCIs) and compare PD performance between the 2 implantation sides and between children with bilateral cochlear implants (BiCIs) and their age-matched peers with normal hearing (NH). DESIGN: All participants completed the Auditory Speech Sound Evaluation Phoneme Discrimination Test. STUDY SAMPLE: The sample included 23 children with SeqBiCIs as the study group and 23 with NH as the control group. RESULTS: A significant difference was found between the scores of the two groups under the CI1 and CI2 conditions (p = 0.001), CI1 and BiCI conditions (p = 0.002), and CI2 and BiCI conditions (p = 0.001). PD scores with CI1 significantly depend on age at CI1 and duration of bilateral use. PD scores with CI1 were significant predictors of PD performance with CI2. Duration of BiCI use was a significant predictor of PD scores with BiCI. CONCLUSIONS: The age at CI1 and the duration of bilateral cochlear implant use were found to improve phoneme discrimination performance in children with a sequential bilateral cochlear implant. According to the success of the CI1, it is possible to predict the success of CI2 use.

Postural Control in Subjects with Incomplete Partition Inner Ear Malformations: A Comparison of Incomplete Partition Types.

INTRODUCTION: Children with inner ear malformation (IEM) are at risk of vestibular loss as well as hearing loss. Incomplete partition (IP) anomalies constitute about 41% of all IEMs. This study aimed to investigate the postural control in subjects with the same type of IP on both sides and to compare their results with cochlear implant (CI) users without IEM and healthy peers. METHODS: The study group consists of 17 subjects with the same IP types on both sides and using auditory implants on at least one side, with the following 3 groups: 6 IP-I subjects (mean age 12.28 ± 6.25), 6 IP-II subjects (mean age 12.90 ± 3.23), and 5 IP-III subjects (mean age 6.98 ± 3.10). Six unilateral CI users (mean age 11.38 ± 3.57) with normal inner ear structures were included in the CI control group, and 6 healthy peers (10.20 ± 4.79) were included in the healthy control group. The postural control was measured using the Bruininks-Oseretsky Test of Motor Proficiency Second Edition (BOT-2) balance subtest. All devices were turned off during the balance test. RESULTS: The BOT-2 balance scale scores were observed to be significantly different between the IP-I and healthy control group (medians of balance scores being 3.00 and 16.00, respectively, p < 0.001) and the IP-III and healthy control group (medians of balance scores being 6.60 and 16.00, respectively, p = 0.04). The IP-II group had better balance scores (median = 8.00) than those of the other IP groups, although there were no significant differences between the IP-II and other groups (p > 0.05). CONCLUSION: This study demonstrated that subjects with the same IP type on both sides and with early implantation may differ in terms of their postural control abilities depending on their IP type. Subjects with IP should be regularly followed up by the vestibular assessment and supported by their postural control ability by vestibular rehabilitation.

3 Tesla MR imaging of the large endolymphatic duct and sac anomaly with audiological correlation.

PURPOSE: Large endolymphatic duct and sac (LEDS) is one of the most common imaging abnormalities in congenital sensorineural hearing loss and is frequently seen with coexistent cochlear anomalies, especially incomplete partition type II. However, MRI findings of accompanying cochlear and especially modiolar dysplasias may be subtle. The purpose of this study is to evaluate the imaging findings of LEDS with high-resolution imaging at 3 T and correlate with the audiological data. METHODS: 3 T temporal bone MRIs of 54 ears in 30 LEDS patients were retrospectively evaluated. The cochlear dysmorphism and modiolar deficiency were assessed qualitatively and quantitatively. The severity of LEDS anomaly, the signal changes within the LEDS and cochlea were also noted. The imaging findings were correlated to the audiological data. RESULTS: The cochlea was abnormal in 77.8% of the ears with an isolated modiolar deficiency in 11.1%. Cochlea and modiolus were completely normal in 11.1% of the ears. In 63% of the ears X-distance was increased. T2 hypointensity within LEDS and cochlea were detected in 42.6%, and 7.4% of the ears, respectively. The median diameters of LEDS were higher in ears with severe to profound HL than ears with normal to moderate HL (p < 0.05). The X-distance, presence of T2 hypointensity within LEDS, and diameters of modiolus did not show statistical correlation with the audiographic data. CONCLUSION: High-resolution 3 T imaging of patients with LEDS anomaly revealed a spectrum of cochlear anomalies, but up to 11.1% of the ears had no underlying anomaly despite severe (endolymphatic duct/sac) dilatation and/or profound HL.

Radiological Features and Pathognomonic Sign of Stapes Footplate Fistula in Inner Ear Malformations.

OBJECTIVE: Some inner ear malformations may cause recurrent meningitis, which may be fatal. The etiology is usually a stapes footplate fistula which enables microorganisms to pass into the inner ear containing cerebrospinal fluid (CSF), causing repeated attacks of meningitis. Radiological signs of the fistula are not obvious and are not reported in detail in the literature. The aim of the study is to investigate the radiological features of stapes footplate fistula in inner ear malformations. METHODS: Radiological findings were analyzed for seventeen patients with inner ear malformations (IEMs) operated on because of recurrent meningitis. Using this information, images of 1,010 patients with IEMs were retrospectively reviewed to investigate the radiological findings of stapes footplate fistula and their relationship to IEMs. They were classified according to the Sennaroglu classification system, and according to different stages of stapes footplate fistula. RESULTS: In the case of a stapes footplate cyst, computerized tomography shows an opacity at the oval window. On magnetic resonance imaging, a fluid filled cystic structure continuous with and having similar signal characteristics to the CSF in the inner ear is a pathognomonic finding of a stapes footplate cyst. It is most commonly found in common cavity anomaly (18.2%); the second most frequent finding is incomplete partition type I (15%). And it can even be seen in cases of cochlear aplasia where only the vestibule is present. CONCLUSION: If the history reveals recurrent meningitis, particular attention should be given to the oval window area, where an opacity, cyst or a leaking lesion should be looked for on the imaging. It is the responsibility of the otolaryngologist to notice these findings, and to operate on the patient to prevent further attacks of meningitis.

Stapedotomy in Congenital Fixation with Cochlear Hypoplasia: A New Concept Among the Treatment Options for Cochlear Hypoplasia.

OBJECTIVES: To introduce the concept of stapedotomy as a new treatment alternative in cochlear hypoplasia (CH) and propose a new guideline for its management. METHODS: Forty-two primary cases out of 355 presented with congenital stapes fixation between January 2003 and September 2015 were included in the study. Computed tomography scans of all cases with congenital stapes fixation were reviewed, and cases with inner ear anomalies were taken into account. Eleven cases had various inner ear anomalies, and 9 cases had various types of CH. In the present paper, only the CH cases with stapes fixation, all of whom underwent stapedotomy, are reviewed regarding preoperative audiological and radiological characteristics as well as surgical findings and postoperative audiological results. RESULTS: The patients were aged between 4 and 22. There were 2 males (3 ears) and 4 females (6 ears). Three cases had bilateral stapedotomy. The remaining 3 cases had unilateral surgery. The average preoperative air-bone gap (ABG) was 50.3 dB. Postoperative hearing: preoperative ABG was 50.3 dB. Postoperative ABG was calculated as 20.1 dB hearing. CONCLUSION: Hearing loss (HL) in hypoplastic cochlea demonstrates the full spectrum of HL types. CH is a unique inner ear anomaly that can be treated with all of the available rehabilitation modalities. As a result of current findings, a new treatment algorithm for CH is proposed.

Unilateral Facial Paralysis and Ophthalmoplegia Caused by Lower Lip Carcinoma: A Case Report.

We discuss a case of lower lip carcinoma which presented with atypical symptoms; facial paralysis, conductive type hearing loss, and ophthalmoplegia. Due to an earlier resection, no mass was evident on the primary examination. Diagnostic imaging revealed a mass originating from the lower lip, the perineural spread of the tumor along the left inferior alveolar nerve to the left infratemporal fossa and the left foramen ovale. Through a retrograde course from the foramen ovale, the tumor extended the ipsilateral cavernous sinus, Meckel's cave, and cisternal portion of the CN V. This atypical spread pattern of the tumor caused symptoms that may be attributed to a diagnosis related to the ear. The biopsy confirmed squamous cell carcinoma, and the patient was referred for chemotherapy and radiotherapy.

Children with Auditory Brainstem Implant: How Do They Perform in Motor and Language Skills?

BACKGROUND: Young children are able to explore new objects and practice language through the acquisition of motor skills that lead to their overall development. Congenital hearing loss and total vestibular loss may contribute to the delay in speech and motor skill development. OBJECTIVES: To investigate the relationship between motor development performance, speech perception, and language performance in children with auditory brainstem implant (ABI). METHOD: Ten children, aged 4-17 years (mean age 9.76 ± 4.03), fitted with unilateral ABI for at least 2 years due to the presence of labyrinthine aplasia and rudimentary otocyst at least 1 side were included in the study. Several standardized tests, such as Bruininks-Oseretsky Motor Proficiency Test-2 (BOT-2), Children's Auditory Perception Test Battery, Meaningful Auditory Integration Scale (MAIS), and Test of Early Language Development-3, were performed to evaluate their skills of fine motor control, balance, manual dexterity, language, and auditory perception. RESULTS: A significant correlation was established between the BOT-2 manual dexterity and MAIS scores (r = 0.827, p < 0.05) and between the manual dexterity and language skills (for expressive language, r = 0.762, p < 0.05; for receptive language, r = 0.650, p < 0.05). Some of the BOT-2 balance tasks, such as standing on 1 leg on a line with eyes closed, standing on 1 leg on a balance beam with eyes open, standing heel-to-toe on a balance beam, and walking forward heel-to-toe on a line, showed a strong correlation with their receptive and expressive language performance (p < 0.05). CONCLUSION: The current study has indicated that significantly poor manual and balance performances are associated with poor speech perception and language skills in children with ABI. The authors recommend performing a vestibular assessment before and after ABI surgery and the use of a holistic rehabilitation approach, including auditory and vestibular rehabilitation, to support development of the children with ABI.