The Relationship of Vestibulo-Ocular Reflex With Self-Reported Dizziness Handicap in Patients With Vestibular Deafferentation.

The relationship between objective vestibular tests and subjective vestibular tests is a controversial topic. In this study, to contribute to this issue, the vestibulo-ocular reflex features and their relationship with balance perception at long-term follow-up in vestibular neurectomy (VN) and total labyrentectomy patients were evaluated. Prospectively, 19 VN and 18 labyrinthectomy patients were enrolled in this study. Patients underwent video head impulse test (VHIT) as objective vestibular test and dizziness handicap inventory (DHI) as subjective vestibular test when they attended to their control visit follow-up between March and September 2017. Lateral canal corrective saccades were classified as organized pattern and deorganized (scattered) pattern. In our results, the saccade pattern analysis (between organized and deorganized saccades) regarding the DHI scores gave P value as .039 for covert saccade pattern and .050 for overt saccade pattern. Therefore, we conclude that the presence of saccades, their patterns, and amplitudes provide extra information at assessing the results of the VHIT test, and the organized pattern of saccades is related to a stable vestibular system and better balance perception.

Temporal bone paragangliomas: 15 years experience / Paraganglioma do osso temporal: 15 anos de experiência

Abstract Introduction Temporal bone paragangliomas (TBPs) are benign tumors arising from neural crest cells located along the jugular bulbus and the tympanic plexus. In general surgical excision, radiotherapy and wait-and-scan protocols are the main management modalities for TBPs. Objective In this paper we aim to present our clinical experience with TBPs and to review literature data. Methods The patients who were operated for tympanomastoid paraganglioma (TMP) or tympanojugular paraganglioma (TJP) in our clinic in the last 15 years were enrolled in the study. A detailed patient's charts review was performed retrospectively. Results There were 18 (52.9%) cases with TMPs and 16 (47.1%) cases with TJPs, a total of 34 patients operated for TBPs in this time period. The mean age was 50.3 ± 11.7 (range 25-71 years). The most common presenting symptoms were tinnitus and hearing loss for both TMPs and TJPs. Gross total tumor resection was achieved in 17 (94.4%) and 10 (62.5%) cases for TMPs and TJPs, respectively. Five patients (31.2%) with TJP experienced facial palsy following the operation. For all the patients the mean follow-up period was 25.8 months (range 4-108 months). Conclusion In conclusion, based on our findings and literature review, total surgical excision alone or with preoperative embolization is the main treatment modality for TBPs. However radiotherapy, observation protocol and subtotal resection must be considered in cases of preoperative functioning cranial nerves, large tumors and advanced age.

Resumo Introdução Paragangliomas do osso temporal (POT) são tumores benignos derivados de células da crista neural localizados ao longo do bulbo jugular e do plexo timpânico. Em geral, a excisão cirúrgica, a radioterapia e os protocolos de acompanhamento com estudos por imagem são as principais modalidades de conduta para o POT. Objetivo Apresentar nossa experiência clínica com POT e revisar os dados da literatura. Método Os pacientes que foram submetidos a cirurgia para paraganglioma timpanomastoideo (PTM) ou paraganglioma timpanojugular (PTJ) em nossa clínica nos últimos 15 anos foram incluídos no estudo. Realizou-se retrospectivamente uma revisão detalhada dos prontuários dos pacientes. Resultados Houve 18 (52,9%) casos com PTM e 16 (47,1%) com PTJ, portanto, um total de 34 pacientes operados para POT nesse período. A idade média foi de 50,3 ± 11,7 (intervalo de 25-71 anos). Os sinais e sintomas de apresentação mais comuns foram o zumbido e perda auditiva, tanto para PTM quanto para PTJ. A ressecção tumoral completa foi obtida em 17 (94,4%) e 10 (62,5%) casos para PTM e PTJ, respectivamente. Cinco pacientes (31,2%) com PTJ apresentaram paralisia facial decorrente da cirurgia. Para todos os pacientes o tempo médio de seguimento foi de 25,8 meses (intervalo 4-108). Conclusão Com base nos nossos dados e na revisão da literatura, a excisão cirúrgica total isolada ou com embolização pré-operatória é a principal modalidade de tratamento para POT. No entanto, a radioterapia, o protocolo de observação e a ressecção subtotal devem ser considerados no caso de nervos cranianos funcionais no pré-operatório, grandes tumores e idade avançada.

Visual and Postural Motion-Evoked Dizziness Symptoms Are Predominant in Vestibular Migraine Patients.

Background: Vestibular migraine (VM) is one of the most common underdiagnosed disorders. We aimed to study the clinical characteristics of VM patients who were referred to a neurology-headache unit by otolaryngology after exclusion of peripheral causes of vertigo. Methods: One hundred and one patients diagnosed with VM in the headache unit were included. Description of vestibular symptoms, demographic and clinical features, trigger factors, accompanying diseases, and response to vestibular-suppressant medications and prophylactic migraine treatment were evaluated. Results: Vestibular symptoms were triggered by daily head and body movements and mainly consisted of brief attacks lasting seconds (60.4% of patients) although the total duration of the vestibular episode lasted hours or days. Other aggravating factors were moving visual stimuli, passive motion, and visually busy environments. Visually induced vestibular symptoms were defined by 71.3% of the patients, and positional motion-induced vestibular symptoms were described by 82.2% of the patients. Vestibular symptoms were mainly defined as feeling the ground slipping from under their feet (40.6%), feeling like there is an earthquake or swaying (27.7%), sensation of rocking on a boat (26.7%), and sensation as if stepping on empty space (24.8%). The majority of the patients (83.2%) previously used vestibular-suppressant drugs, and these drugs were effective temporarily only in 12.9%. Conclusions: Chronic recurrent dizziness symptoms, rather than internal or external vertigo, are predominant in our VM patients. Recurrent brief dizziness attacks induced upon routine visual and/or postural motion, longstanding symptoms with limited response to vestibular suppressants, and precipitation by typical migraine triggers are suggestive of VM.

Temporal bone paragangliomas: 15 years experience.

INTRODUCTION: Temporal bone paragangliomas (TBPs) are benign tumors arising from neural crest cells located along the jugular bulbus and the tympanic plexus. In general surgical excision, radiotherapy and wait-and-scan protocols are the main management modalities for TBPs. OBJECTIVE: In this paper we aim to present our clinical experience with TBPs and to review literature data. METHODS: The patients who were operated for tympanomastoid paraganglioma (TMP) or tympanojugular paraganglioma (TJP) in our clinic in the last 15 years were enrolled in the study. A detailed patient's charts review was performed retrospectively. RESULTS: There were 18 (52.9%) cases with TMPs and 16 (47.1%) cases with TJPs, a total of 34 patients operated for TBPs in this time period. The mean age was 50.3± 11.7 (range 25-71 years). The most common presenting symptoms were tinnitus and hearing loss for both TMPs and TJPs. Gross total tumor resection was achieved in 17 (94.4%) and 10 (62.5%) cases for TMPs and TJPs, respectively. Five patients (31.2%) with TJP experienced facial palsy following the operation. For all the patients the mean follow-up period was 25.8 months (range 4-108 months). CONCLUSION: In conclusion, based on our findings and literature review, total surgical excision alone or with preoperative embolization is the main treatment modality for TBPs. However radiotherapy, observation protocol and subtotal resection must be considered in cases of preoperative functioning cranial nerves, large tumors and advanced age.

Histopathological Effects of Parylene C (poly-chloro-p-xylylene) in the Inner Ear.

OBJECTIVE: To assess the histopathological effects of parylene C (PC) (poly-chloro-p-xylylene) in the inner ear. METHODS: Nine adult Dunkin Hartley guinea pigs (500-600 g) were included in the study. PC pieces were inserted into the cochlea in the right ear of the animals (study group). The round windows were punctured in the left ears comprised the control group. After three months, the animals were sacrificed, and the dissected temporal bones were examined under a light microscope. RESULTS: No significant difference was revealed between the study and control groups regarding histopathological findings such as perineural congestion, perineural inflammation, neural fibrosis, number of ganglion cells, edema, and degeneration of ganglion cells (p>0.05). CONCLUSION: PC did not cause any additional histopathologic damage in the cochlea. This finding may be promising regarding the use of PC in cochlear implant electrodes as an alternative to silicon materials in the future.

The effects of sodium-2-mercaptoethanesulfonate application on the neural and neurovascular tissues: An experimental animal study.

BACKGROUND: Sodium-2-mercaptoethanesulfonate (MESNA) is a protective agent that is also used as "a chemical dissector" in various surgical fields. The aim of this study is to evaluate the toxic effects of MESNA on neural and neurovascular structures based on a morphological analysis and examine its safety in neurotological applications. METHODS: Three groups of guinea pigs were used as subjects. MESNA solution (50 and 100%) and saline solution were applied to the subarachnoid space over the brain tissue via a middle fossa approach of study and control groups, respectively. Effects of MESNA were assessed by means of light microscope. McNemar Chi-square test was used to evaluate the histopathological findings. Statistical significance of P < 0.05 was taken as criterion. RESULTS: No morphological changes were observed on vascular and neural structures in the study groups in both concentrations, compared to the control group. CONCLUSIONS: On a morphological basis, a single application of MESNA does not cause any morphological changes that indicate a toxicity in neural and neurovascular structures.

Effects of polylactic acid film on middle ear mucosa and cochlear function in Guinea pigs.

Our aim was to assess the effects of polylactic acid (PLA) on middle ear mucosa and cochlea, to be used as a film barrier for postoperative adhesion prevention in the middle ear. Twenty-one albino Guinea pigs were included in the study. A window was opened on both tympanic bulla and on one side PLA material was placed in the middle ear and on the other side only fenestration was performed and used as a control. All Guinea pigs underwent evaluation of tympanic membranes microscopically; functional hearing was analyzed by auditory brainstem responses preoperatively, in the first and the sixth month. All Guinea pigs were killed on the sixth month for histopathologic evaluation of their temporal bones. There was no statistical difference between both groups regarding hearing thresholds, interpeak wave latencies preoperatively and on first and the sixth months postoperatively. Histopathological evaluation revealed no specific changes. There was a mild local inflammation both in the PLA implanted and control ears. PLA film barrier most likely has no toxic effects on Guinea pig middle ear and does not show any ototoxic side effects.

[Vascular compression syndromes: our 24 year endoscope-assisted microvascular decompression experiences]. / Vasküler kompresyon sendromlari: 24 yillik endoskop yardimli mikrovasküler dekompresyon deneyimlerimiz.

OBJECTIVES: This study aims to report our experience on endoscope-assisted microvascular decompression experiences performed due to vascular compression syndromes such as hemifacial spasm, trigeminal neuralgia, and cochleovestibular nerve compression. PATIENTS AND METHODS: Between March 1999 and June 2013, 55 patients (34 females, 21 males; mean age 44 years; range 24 to 77 years) underwent endoscope-assisted microvascular decompression surgery through a retrosigmoid approach due to vascular compression syndromes in our clinic. The diagnosis was based on history, neurological examination, magnetic resonance imaging findings and audio-vestibular tests. RESULTS: A total of 49 patients (89.1%) had complete relief of the symptoms and two had (3.6%) a partial relief, while four had (7.3%) no relief of the symptoms. Only two patients had (3.6%) cerebrospinal fluid leakages as a perioperative complication. The major offending vessels were anterior and inferior cerebellar arteries in 14 patients. Four patients with essential hypertension became normotensive after decompression of the left medulla oblongata as well. CONCLUSION: Microvascular decompression surgery provides a significant relief of the symptoms in patients with vascular compression syndromes. An angled endoscope as an adjunct to microscope contributes to the diagnosis of the offending vessel in the root entry zone of the cranial nerve.

Methods and preliminary outcomes of pediatric auditory brainstem implantation.

OBJECTIVE: The objective was to provide information about methods used and preliminary outcomes for pediatric ABI (auditory brainstem implant). STUDY DESIGN: An analysis of outcome was performed in children who received an ABI. METHODS: Twelve children received a MED-EL ABI system. Progress in audition and language was monitored through parental reports, questionnaires, profiles, and closed-set tests. RESULTS: The median number of active electrodes was 9 of 12. Seven of 12 users consistently respond to sound, and 5 of 12 do not. Highest performers can recognize words in small sets and have begun to use some words. CONCLUSION: Auditory brainstem implants appear to be beneficial for some pediatric patients who cannot benefit from traditional cochlear implant surgery. Benefits in the short-term can be recognition of environmental sounds, recognition of some words and very commonly used phrases, and the beginning use of words. Although some of our ABI users demonstrate no response to sound, they do want to wear their sound processors all waking hours. The cause of lack of response may be related to the second intervention, which might have led to displacement of the electrode array, or presence of additional handicaps or syndromes. However, the results are less than optimal. The relatively short postoperative follow-up duration is a considered weakness of this study.

Quantitative measurement of m-RNA levels to assess expression of cyclooxygenase-II, inducible nitric oxide synthase and 12-lipoxygenase genes in middle ear cholesteatoma.

To assess expression of three main inflammatory genes, COX-II, ALOX-12 and i-NOS, quantitatively at transcriptional level in cholesteatoma matrix tissue. Ten patients who have chronic otitis media with primary acquired cholesteatoma were included in this study. Tissue samples obtained from cholesteatoma matrix and external ear canal skin (control tissue). Expression of the targeted genes (COX-II, i-NOS and LOX-12) was assessed using real-time quantitative polymerase chain reaction (RT-PCR) technique. The amount of COX2 mRNA was significantly higher in cholesteatoma matrix at transcriptional level (p = 0.038). There was no statistically significant difference regarding expression of iNOS and LOX12 mRNA levels (p > 0.05). There is a significant overexpression of the mRNA of COX-II in cholesteatoma matrix, which indicates a difference between the normal skin and cholesteatoma matrix at molecular level. COX-II gene overexpression seems to be associated with pathogenesis of cholesteatoma. This molecular change is similar to the molecular abnormalities observed in some benign and malignant neoplasms. Invasive and locally destructive nature of cholesteatoma may be due to COX-II overexpression. Absence of an increase in the gene expressions of i-NOS and LOX-12 in cholesteatoma matrix suggests that these mediators may not be related with the pathogenesis and evolution of cholesteatoma.

An analysis of petrous bone cholesteatomas treated with translabyrinthine transotic petrosectomy.

CONCLUSION: In cases of petrous apex cholesteatoma, radical removal should be prioritized over an unreasonable sparing of hearing or facial symmetry. Restoration of facial nerve function is achievable by reanimation procedures. OBJECTIVES: To analyze clinical manifestations, surgical findings, and postoperative functional results of petrous apex cholesteatoma. METHODS: From 1995 to 2012, 34 cases of petrous apex cholesteatoma underwent operations. Clinical and surgical findings and postoperative functional outcomes were analyzed retrospectively. RESULTS: Hearing loss was the most common symptom in 95% of patients, followed by otorrhea in 64% and facial palsy in 59%. Four patients had recurrent facial palsy. In eight (24%) patients petrous apex cholesteatomas were recurrent or iatrogenic in origin. The supralabyrinthine and massive type of petrous bone cholesteatoma were the most common types, followed by, infralabyrinthine-apical, infralabyrinthine, and apical. Among 18 cases with facial nerve paralysis, 8 underwent hypoglossal-facial nerve anastomosis, 4 underwent rerouting and end to end anastomosis, 3 of them did not undergo any treatment because of the duration of facial palsy (> 3 years), and another 3 patients for whom we had recommended facial-hypoglossal anastomosis did not accept the operation. There were no major complications. Recurrence was observed in two (5%) cases.

Audiological correlates of tumor parameters in acoustic neuromas.

The objective of this study was undertaken to evaluate a correlation of pure tone audiometry, speech audiometry with tumor size, the presence of intrameatal extension, contralateral pure tone audiometry, and contralateral speech audiometry results at acoustic neuroma (AN) patients. Seventy-six patients with a unilateral-cerebellopontine angel tumor with/without internal acoustic canal (IAC) were included in this study. Data on pure tone audiometry, speech discrimination scores (SDS), speech reception thresholds (SRT), tumour size, and extension to IAC were analyzed retrospectively. As a result, patients with unilateral ANs had an asymmetric sensorineural hearing loss and had an asymmetric finding on the results of speech audiometry was present. We could not find any correlation between the size of the tumor and the hearing levels at each frequency. In addition, there appeared to be no significant correlation between the extension of tumor to the IAC and the audiological findings.

Functional outcomes of Vibrant Soundbridge applied on the middle ear windows in comparison with conventional hearing aids.

CONCLUSION: Vibrant Soundbridge (VSB) application to the middle ear windows yields better functional outcomes than conventional hearing aids. However, speech discrimination scores obtained with VSB and conventional hearing aids are similar. OBJECTIVE: To assess audiological outcomes of round and oval window applications of VSB in comparison with conventional hearing aids. METHODS: Nineteen adult patients were included in the study. The patients had mild to moderate, moderate or moderate to profound conductive or mixed hearing loss. During surgery the floating mass transducer (FMT) was placed on the round (n = 14) or oval (n = 5) window. After the surgery, audiometric evaluation and free field audiometric evaluation of both ears was carried out. RESULTS: The hearing thresholds in the low frequencies were not significantly different between the conventional hearing aids and VSB. The functional gains obtained with oval and round window approaches were similar except for 500 Hz. The hearing thresholds in the mid and high frequencies were significantly better with VSB than the conventional hearing aids. The functional gain in the low frequencies was not significantly different between VSB and conventional hearing aids. The functional gain in the other frequencies was significantly better with VSB than conventional hearing aids.

Complications of pediatric auditory brain stem implantation via retrosigmoid approach.

OBJECTIVE: We aimed to present the complications of auditory brain stem implantations (ABI) in pediatric patients which were performed via retrosigmoid approach. METHODS: Between March 2007 and February 2010, five prelingually deaf children underwent ABI (Medel device) operation via retrosigmoid approach. All children had severe cochlear malformations. The ages ranged from 20 months to 5 years. The perioperative complications encountered in 2 patients were evaluated retrospectively. RESULTS: No intraoperative complication was observed in the patients. Cerebrospinal fluid (CSF) leakage was the most common postoperative complication that was seen in 2 patients. The CSF leak triggered a cascade of comorbidities, and elongated the hospitalization. CONCLUSION: Pediatric ABI surgery can lead to morbidity. The CSF leak is the most common complication encountered in retrosigmoid approach. The other complications usually result from long-term hospital stay during treatment period of the CSF leak. Therefore, every attempt must be made to prevent occurrence of CSF leaks in pediatric ABI operations.

Otoacoustic emissions and effects of contralateral white noise stimulation on transient evoked otoacoustic emissions in diabetic children.

OBJECTIVE: In this study, our aim was to determine presence of dysfunction in the efferent auditory system of children with type-I diabetes mellitus (DM) presenting no evidence of symptomatic neuropathy. METHODS: Thirty children with type-I DM (DM group) and 31 age matched healthy children (control group) with normal hearing and middle ear function were entered to the study. Distortion product otoacoustic emissions (DPOAE), transiently evoked otoacoustic emissions (TEOAE), and spontaneous otoacoustic emissions (SOAE) measurements were performed. Then, the TEOAE recording was repeated while a continuous broadband white noise (bandwidth: 50-8000 Hz) presented at 40 dB SL was delivered to the contralateral ear for efferent auditory system suppression. RESULTS: We found that contralateral stimulation (CS) with white noise resulted in significantly more pronounced suppression of the TEOAE response amplitude in healthy controls compared to DM group at 2000 and 4000 Hz frequencies. Further, a relatively higher percentage of the controls had suppression in at least three frequencies compared to DM group. SOAE prevalence was found to be higher in the DM group. CONCLUSIONS: Our findings suggest presence of a dysfunction in medial olivocochlear efferent system in diabetic children. This may be regarded as an early central manifestation of diabetic neuropathy.

Management of cerebrospinal fluid leak following retrosigmoid posterior cranial fossa surgery.

OBJECTIVE: To detail our experience in the management of cerebrospinal fluid (CSF) leak following posterior cranial fossa surgery by the retrosigmoid approach. PATIENTS AND METHODS: 412 patients who underwent posterior cranial fossa surgery by the retrosigmoid approach for a variety of diseases were included in the study. RESULTS: There were 32 CSF leaks (7.7%) in the 412 patients. Of these, 16 were leaks from the incision site (50%) and 16 (50%) were CSF rhinorrheas. The CSF leaks were documented in 22 of the vestibular schwannoma surgeries (68.7%) and 10 of the vestibular nerve sectioning surgeries (31.3%). No CSF leak was seen following microvascular decompression and auditory brain stem implantation surgeries. Ten patients could be treated conservatively. Twelve patients needed the placement of a lumbar drainage. Surgical reexploration was performed in 10 patients. CONCLUSIONS: Initially, a conservative treatment should be instituted in the case of a CSF leak. If the conservative treatment fails, the placement of a lumbar drainage should be considered. Patients that do not respond to lumbar drainage require surgical reexploration. The abovementioned algorithm represents the safest and best option in the presence of a CSF leak, as confirmed by the absence of recurrences or multiple revisions in this study.

GJB2 and mitochondrial A1555G gene mutations in nonsyndromic profound hearing loss and carrier frequencies in healthy individuals.

This study aimed to assess mutations in GJB2 gene (connexin 26), as well as A1555G mitochondrial mutation in both the patients with profound genetic nonsyndromic hearing loss and healthy controls. Ninety-five patients with profound hearing loss (>90 dB) and 67 healthy controls were included. All patients had genetic nonsyndromic hearing loss. Molecular analyses were performed for connexin 26 (35delG, M34T, L90P, R184P, delE120, 167delT, 235delC and IVS1+1 A-->G) mutations, and for mitochondrial A1555G mutation. Twenty-two connexin 26 mutations were found in 14.7% of the patients, which were 35delG, R184P, del120E and IVS1+1 A-->G. Mitochondrial A1555G mutation was not encountered. The most common GJB2 gene mutation was 35delG, which was followed by del120E, IVS1+1 A-->G and R184P, and 14.3% of the patients segregated with DFNB1. In consanguineous marriages, the most common mutation was 35delG. The carrier frequency for 35delG mutation was 1.4% in the controls. 35delG and del120E populations, seems the most common connexin 26 mutations that cause genetic nonsyndromic hearing loss in this country. Nonsyndromic hearing loss mostly shows DFNB1 form of segregation.