Ramsay Hunt syndrome: Clinical analysis of 15 cases

The Ramsay Hunt syndrome (RHS) is characterized by acute facial nerve paresis and/or paralysis accompanied by a herpetic lesion on the external ear. Fifteen patients were evaluated and treated for RHS. After a detailed history was obtained, clinical examination was performed, and treatment was initiated based on steroids and acyclovir. The House-Brackmann grading system (HB I-VI) was used to evaluate the facial nerve function; this was further electrophysiologically assessed with the nerve excitability test (NET) and electro-neurography. In RHS cases, the most common accompanying findings were the cochleovestibular symptoms (73%) and in particular vertigo and balance disorder. The combined treatment of acyclovir with steroids resulted in satisfactory facial nerve function (HB I-II) in 75% of cases. The recovery was satisfactory in all the cases in which the NET was normal or diminished. In 4 cases in which the recovery was non-satisfactory (ΗΒΙΙΙ-V) no response was initially obtained in NET. In RHS, the possible neuritis and the inflammatory process which occur into the internal auditory canal may result in facial nerve dysfunction and cochleovestibular symptoms. The combined therapy of the antiviral agent acyclovir with the anti-inflammatory effect of steroids is recommended for the treatment of RHS. The NET was proved as the most useful method in the prognostication of RHS.The Ramsay Hunt syndrome (RHS) is characterized by acute facial nerve paresis and/or paralysis accompanied by a herpetic lesion on the external ear. Fifteen patients were evaluated and treated for RHS. After a detailed history was obtained, clinical examination was performed, and treatment was initiated based on steroids and acyclovir. The House-Brackmann grading system (HB I-VI) was used to evaluate the facial nerve function; this was further electrophysiologically assessed with the nerve excitability test (NET) and electro-neurography. In RHS cases, the most common accompanying findings were the cochleovestibular symptoms (73%) and in particular vertigo and balance disorder. The combined treatment of acyclovir with steroids resulted in satisfactory facial nerve function (HB I-II) in 75% of cases. The recovery was satisfactory in all the cases in which the NET was normal or diminished. In 4 cases in which the recovery was non-satisfactory (ΗΒΙΙΙ-V) no response was initially obtained in NET. In RHS, the possible neuritis and the inflammatory process which occur into the internal auditory canal may result in facial nerve dysfunction and cochleovestibular symptoms. The combined therapy of the antiviral agent acyclovir with the anti-inflammatory effect of steroids is recommended for the treatment of RHS. The NET was proved as the most useful method in the prognostication of RHS

Cornelia De Lange Syndrome and Cochlear Implantation

Introduction: Literature regarding the different degrees of hearing loss in patients with Cornelia de Lange syndrome (CDLS) reports that half of the affected patients exhibit severe to profound sensorineural hearing loss. We present the first pre-school child with CDLS who underwent cochlear implantation for congenital profound sensorineural hearing loss.   Case Report: A 3-year-old boy with CDLS underwent unilateral cochlear implantation for bilateral profound sensorineural hearing loss. He had characteristic facial features, bushy eyebrows and synophrys, limb anomalies, growth and mental retardation. Based on the results of postoperative speech perception and production tests, his gain in language skills and expressive vocabulary was modest. However, a cochlear implantation had a significant effect on auditory development, in terms of making him aware of sound localization and the different types of environmental sound.   Conclusion: Criteria for cochlear implantation are expanding and now include children with disabilities in addition to deafness, such as those with CDLS. Profoundly hearing-impaired children affected by borderline mental retardation should be considered as potential candidates for cochlear implantation

Video Head Impulse Test (vHIT): Value of Gain and Refixation Saccades in Unilateral Vestibular Neuritis

The aim of this study was to evaluate gain and refixation saccades (covert and overt) using a video head impulse test (vHIT) in the horizontal and vertical planes in patients after the onset of unilateral acute vestibular neuritis (AVN). Thirty-five patients were examined in the acute stage of AVN and at follow-up (range, 6–30 months); a control group of 32 healthy subjects also participated. At onset, the mean gain was significantly lower on the affected side in all of the semi-circular canal planes, mainly in the horizontal canal plane, and saccades (covert and overt) were more prevalent in the horizontal compared to the vertical canal planes. Multi-canal affection occurred more frequently (80% for gain, 71% for saccades) than isolated canal affection. At follow-up, which ranged from 6 to 30 months, the gain was recovered in all of the canals (anterior in 50%, horizontal in 42.8%, and posterior canal in 41.1% of cases), while covert and overt saccades were reduced in the horizontal and vertical planes. However, covert saccades were still recorded in a greater proportion (69%) than overt saccades (57%) in the horizontal plane and at a lower rate in the vertical planes. The compensatory mechanisms after AVN mainly involve the horizontal canal, as the refixation saccades—especially covert ones—were more frequently recorded in the horizontal than vertical canals

Hyperbaric Oxygen Therapy in the Treatment of Sudden Sensorineural Hearing Loss

Sudden sensorineural hearing loss (SSHL) is a frustrating and frightening experience for a patient [...

The Effect of Anxiolytics on Tinnitus

Tinnitus is a perceptual disorder in which sound is perceived by the patient in the absence of an external or internal acoustic stimulation [...

Autoimmune Hearing Loss: A Diagnostic Challenge

Autoimmune hearing loss (AIHL) is a clinical disease and may involve the deposition of immune complexes in the labyrinth vessels, the activation of the complement system, the functional alteration in T-cell subpopulations, or an inflammation process in the inner ear [...

Socio-Economic Status and Language Development in Hearing Loss: A Critical Appraisal

The impact of language input on children’s speech, language, and brain development was borne out of Hart and Risley’s famous “30-million-word gap”. A perspective bolstered by many studies in the last decade relates higher socio-economic status (SES) to better qualitative and quantitative differences in children’s speech. The logic chains found in these studies suggest that literacy development depends on language and brain development. Thus, brain building develops based on environmental experience and language input depends on the brain’s perception of the auditory information. This essay uses the latest published peer-reviewed research to outline the current landscape of the role of SES in the development of speech and language skills among children with hearing loss (HL) who are enrolled in auditory-driven habilitation programs. This essay argues that low SES families may provide sufficient input for their children. The outcome of auditory-driven programs implemented by speech-language pathologists (SLPs) seems to be detached from SES. The role of SES on this developmental trajectory remains unclear, and clinical practice may be related to other validated and robust parameters related to hearing loss

How effective Auditory-Verbal Therapy (AVT) is for building language development of children with cochlear implants? A systematic review.

This systematic review was designed to investigate the effectiveness of Auditory-Verbal Therapy (AVT) based on research findings of the last ten years. The systematic review was designed based on PRISMA guidelines. Search terms were chosen based on the research question and used in a search on PubMed database. Last decade’s published peer-reviewed papers meeting inclusion criteria were reviewed based on. The results revealed AVT as an important clinical approach that improves young cochlear implant (CI) children to outperform peers in bilingual-bicultural pro-grams in receptive vocabulary and speech perception or at the least be at a similar level on speech, language and self-esteem. Other aspects related with voice seemed also benefited, placing young CIs in the normal range for receptive vocabulary development. Less improvement noted in the ar-ea of reading. AVT approach can positively assist infants develop spoken language and support full integration into mainstream society despite the limited evidence presented. This position is supported by research findings of young CIs comparable to their hearing peers. Overall studies suggest AVT as a positive clinical approach for spoken language of young CIs and provide evi-dence that there is no advantage for the use of other alternative communication models before or after CI

Utility of otoacoustic emissions and olivocochlear reflex in predicting vulnerability to noise-induced inner ear damage

Aim: The aim of the present study was to explore the possible utility of otoacoustic emissions (OAEs) and efferent system strength to determine vulnerability to noise exposure in a clinical setting. Materials and Methods: The study group comprised 344 volunteers who had just begun mandatory basic training as Hellenic Corps Officers Military Academy cadets. Pure-tone audiograms were obtained on both ears. Participants were also subjected to diagnostic transient-evoked otoacoustic emissions (TEOAEs). Finally, they were all tested for efferent function through the suppression of TEOAEs with contralateral noise. Following baseline evaluation, all cadets fired 10 rounds using a 7.62 mm Heckler & Koch G3A3 assault rifle while lying down in prone position. Immediately after exposure to gunfire noise and no later than 10 h, all participants completed an identical protocol for a second time, which was then repeated a third time, 30 days later. Results: The data showed that after the firing drill, 280 participants suffered a temporary threshold shift (TTS) (468 ears), while in the third evaluation conducted 30 days after exposure, 142 of these ears still presented a threshold shift compared to the baseline evaluation [permanent threshold shift (PTS) ears]. A receiver operating characteristics curve analysis showed that OAEs amplitude is predictive of future TTS and PTS. The results were slightly different for the suppression of OAEs showing only a slight trend toward significance. The curves were used to determine cut points to evaluate the likelihood of TTS/PTS for OAEs amplitude in the baseline evaluation. Decision limits yielding 71.6% sensitivity were 12.45 dB SPL with 63.8% specificity for PTS, and 50% sensitivity were 12.35 dB SPL with 68.2% specificity for TTS. Conclusions: Interestingly, the above data yielded tentative evidence to suggest that OAEs amplitude is both sensitive and specific enough to efficiently identify participants who are particularly susceptible to hearing loss caused by impulse noise generated by firearms. Hearing conservation programs may therefore want to consider including such tests in their routine. As far as efferent strength is concerned, we feel that further research is due, before implementing the suppression of OAEs in hearing conservations programs in a similar manner

Consonant Repertoire of a Prelinguistically Deaf Child with Late-Mapping Cochlear Implants

Objective: The present case study aims to report on the consonant repertoire during the pre-linguistic and first linguistic stage of a Greek-Cypriot speaking child bilaterally implanted with multichannel Cochlear Implants (CIs). Background: Children with Hearing Loss (HL) produce canonical babble later, and consonantal inventories of HL children are smaller. However, the consonant repertoire of CI Greek-speaking children has not been examined thus far and research on types of consonantal errors during phonological acquisition is scant. Clinical Case: A pre-linguistically deaf child (CY, 7;0 years old) received the first CI at 7 months of age, but the external part of the device was fitted at 2;7 years. An investigation of the child’s speech at 7;0 years was conducted through auditory analysis. The child’s canonical utterances were transcribed in IPA and his consonants were classified into subcategories, depending on articulation place, articulation manner and resonance. Regarding place, alveolar consonants were the main category produced. As regards manner, closed consonants was the first category to appear, while in terms of voicing, voiceless consonants were recorded more often than voiced ones. The analysis also showed that consonants /t/, /s/ and /p/ were dominant in the child’s speech and revealed several phonological processes. Conclusion: The present case holds special interest as the child’s phonological system is still between the pre-linguistic/first linguistic stages due to the delayed CI mapping. The results agree in part with several studies in the literature, while specific phonological error patterns observed, remain to be verified in other CI Greek-speaking children