Detailed insight in intraoperative eABR measurements to assist auditory brainstem implantation in a patient with neurofibromatosis type 2.

Auditory brainstem implant (ABI) is used to provide auditory sensations in patients with neurofibromatosis type 2 who lost their hearing due to a surgical removal of the tumor. ABI surgery, implant activation and follow-up sessions present unique challenges including the exact placement of the electrode pad in the lateral recess of the IVth ventricle, identification of electrodes that trigger non-auditory sensation and their deactivation which lowers the number of electrodes responsible for hearing, changes of T- and C-levels across follow-up sessions. We present a complete procedure using an example case starting from the surgical part with the detailed description of intraoperative eABR measurement as a guidance for pad placement to the ABI activation and first fitting sessions with auditory sensation assessment. Since the first ABI electrode pad position presented non-satisfactory intraoperative eABR results it was decided to move the pad slightly which resulted in better eABR (more electrodes with auditory responses). The discussed patient demonstrated great auditory and speech perception results after the first ABI fitting (which included three sessions over 2 consecutive days). Repositioning of the ABI electrode pad during the surgery was carried out taking into account the intraoperative eABR results and this led to an overall positive outcome for the patient. The placement of ABI electrode pad is crucial for later auditory results. This study provides detailed insight in this very specialized procedure that is not performed in every clinic and adds to the knowledge of intraoperative navigation using eABR measurements during ABI surgery.

Cochlear implantation in autistic children with profound sensorineural hearing loss.

INTRODUCTION: Cochlear implants have become the method of choice for the treatment of severe-to-profound hearing loss in both children and adults. Its benefits are well documented in the pediatric and adult population. Also deaf children with additional needs, including autism, have been covered by this treatment. OBJECTIVE: The aim of this study was to assess the benefits from cochlear implantation in deafened children with autism as the only additional disability. METHODS: This study analyzes data of six children. The follow-up time was at least 43 months. The following data were analyzed: medical history, reaction to music and sound, Ling's six sounds test, onomatopoeic word test, reaction to spoken child's name, response to requests, questionnaire given to parents, sound processor fitting sessions and data. RESULTS: After cochlear implantation each child presented other communication skills. In some children, the symptoms of speech understanding were observed. No increased hyperactivity associated with daily use cochlear implant was observed. The study showed that in autistic children the perception is very important for a child's sense of security and makes contact with parents easier. CONCLUSION: Our study showed that oral communication is not likely to be a realistic goal in children with cochlear implants and autism. The implantation results showed benefits that varied among those children. The traditional methods of evaluating the results of cochlear implantation in children with autism are usually insufficient to fully assess the functional benefits. These benefits should be assessed in a more comprehensive manner taking into account the limitations of communication resulting from the essence of autism. It is important that we share knowledge about these complex children with cochlear implants.

[Vestibular function in cochlear implant candidates]. / Narzad równowagi--ocena uszkodzenia w procesie diagnostycznym do operacji wszczepu slimakowego.

INTRODUCTION: The main problem of a deaf patient is per se communication process. Some of the patients, who are candidates to cochlear implantation report vertigo or imbalance in some everyday situations. The relation of patients' complaints to vestibular loss was evaluated. The authors used electronystagmography which was realized in diagnostic process to cochlear implantation. The results come from the patients diagnosed in our department in 2006-2007. In accordance to the literature, the true vertigo, was rather rare. MATERIAL AND METHODS: Medical history, caloric tests as electronystagmography results were evaluated retrospectively in 47 patients qualified for cochlear implantation in 2006-2007. RESULTS: In the study group, 25 patients did not complain of vertigo; with symmetrical calorics in 10 subjects (40%). The true vertigo reported 6 subjects; 8 subjects had imbalance and unsteadiness on walking. In the group of 47 subjects 8 of them complainted the both- vertigo and imbalance. In the ENG of this group the results of symmetrical responces were evaluated in 57% cases and vestibular hypofunction in 43% subjects. In the group with vertigo (6), 4 subjects (66%) had diagnosed unilateral hypofunction, 1 (17%) bilateral areflexion, 1 subject (17%) had symmetrical responses. CONCLUSIONS: The necessity of the confrontation ENG results to additional examination in audiological part of diagnostic process to cochlear implantation is underlined.

[Results of hearing perception after cochlear implantation in patients with auditory neuropathy]. / Ocena percepcji sluchowej po wszczepieniu implantu slimakowego u pacjenta z neuropatia sluchowa.

INTRODUCTION: Results of hearing perception with patient with auditory and visual neuropathy combined with peripheral polyneuropathy one year after cochlear implantation are presented in this article. Visual problems and hearing loss were progressing during recent years leading to social problems and communication disability. Speech discrimination was much worse than could be suspected based on pure tone audiometry. Lack of benefit from hearing aids was also stated. METHODOLOGY: As the examination method free field speech audiometry was used based on Pruszewicz word tests and set of modified speech therapy tests TAPS II. Simple sentences, monosyllables and polysyllables words as well as logotoms perception and identification of music instruments sounds were estimated. MAIN RESULT: Progress of speech intelligibility in monosyllables test according to Pruszewicz in free field speech audiometry was stated, as well as better understanding of sentences, monosyllables and polysyllables words, and more precise recognition of music instruments sounds. MAIN CONCLUSION: In many sensory-neural profound hearing losses as well as auditory neuropathy cases good results may be obtained by cochlear implantation. Early diagnosis of auditory neuropathy is important because of proper management. Lack of benefit from hearing aids in such cases seems to be indication to cochlear implantation. In described case cochlear implantation improved hearing perception one year after using speech processor.

[Auditory neuropathy--new disease]. / Neuropatia sluchowa--nowa jednostka chorobowa.

Auditory neuropathy, a disorder of neural synchrony, is a retrocochlear hearing disorder identified by absence of auditory brainstem responses (ABR) and presence of evoked acoustic otoemissions (EOA). Registration of emissions shows normal function of external cilliary cells. Patients with auditory neuropathy have difficulties to understand speech especially in presence of beckground noise. Normal conversation requires the support of orofacial reading. The authors presents electrophysiological and behavioral tests useful in diagnostic process. An option in treatment of auditory neuropathy is cochlear implantation. Such treatment is indicated when conventional hearing aids doe not help to improve speech understanding.

[Cochlear implantation by lateral petrosectomy]. / Implantacja wszczepów slimakowych przez petrosektomie boczna.

Cochlear implantation by facial recess is nowadays the approach of choice in normal anatomical conditions in the middle and inner ear. When deafness is coincident with chronic otitis media or partial obliteration of the cochlea, others approaches can be applied. Authors presented lateral petrosectomy as an approach for cochlear implantation in two cases. In both patients all the electrodes were inserted. There were any complications in postoperative period. Lateral petrosectomy is nowadays well codified technique. It is possible to apply this approach for cochlear implantation in cases with chronic otitis media. Good access to the cochlea especially to its basal turn allows for good insertion even in partial obliteration of the scala tympani.