Hearing habilitation with auditory brainstem implantation in two children with cochlear nerve aplasia

OBJECTIVE:The aim of this study is to describe the rehabilitative outcomes of pediatric auditory brainstem implant (ABI) users in the Department of Otolaryngology in the Hacettepe University. It was a retrospective study, and all patients' files were reviewed. MATERIALS and METHODS:The data was collected from 41 children who were fitted with ABI between 2005 and 2013. Inclusion criteria for children in our study are profound, congenital bilateral sensory-neural hearing loss with anomalies (such as cochlear, labyrinthine, and cochlear nerve aplasia) and more than one year of auditory experience with ABI. Post-meningitis patients and neurofibromatosis type 2 (NF2) patients were excluded. Auditory perception was evaluated using the Meaningful Auditory Integration Scale (MAIS), Functioning after Pediatric Cochlear Implantation (FAPCI) instrument, Categories of Auditory Performance (CAP), and Children's Auditory Perception Skills Test in Turkish (CIAT). Speech intelligibility was categorized with speech intelligibility rating (SIR), and language development was assessed using the Test of Early Language Development-Third Edition (TELD-3) and Manchester Spoken Language Development Scale (MSLD). RESULTS:All patients gained basic audiological functions and were able to recognize and discriminate sounds by the third month of ABI surgery. According to the duration of ABI use and learning skills, patients revealed development from word identification to sentence recognition level in a wide spectrum. CONCLUSION:Preliminary results indicate that all children have gained basic auditory perception skills. On the other hand, language and speech development data were varying among children. Additional handicaps seemed to slow down progression. Secondary improvement was seen at psychosocial areas with respect to behavioral and social adjustment as well as eagerness to start communication.

Section: Discussionmentioning

confidence: 99%

“…In 2001, Colletti et al [2] reported the first pediatric ABI patient's results that involve environmental sound awareness and speech detection skills. At present, more clinics in Europe and USA have implemented ABI and have enhanced their rehabilitation procedures.…”

Section: Introductionmentioning

confidence: 99%

Recent Rehabilitation Experience with Pediatric ABI Users

Yücel

Aslan²,

Özkan³

et al. 2015

“…Most authors suggest that cochlear implant surgery is not suitable for these cases. Brain stem implantation may be better therapeutic option than cochlear implantation for these patients [7,8] . In a study of Govaerts and et al [6] showed that patients with type I and type IIb aplasia did not auditive perception with their implant and they became non-users.…”

Section: Discussionmentioning

confidence: 99%

Cochlear Implantation in Patient with Cochlear Nerve Deficiency: A Case Report

Sürmelioğlu

Tarkan²,

Özdemir³

et al. 2016

Diagnostic imaging methods are very important for patients with bilateral sensourinoural hearing loss. Magnetic resonance imaging (MRI) is able to demonstrate the vestibulocochlear nerve and facial nerve in the internal acoustic canal. Also computed tomography can be helpful to determination of the deficiency of the cochlear nerve. Cochlear nerve anomalies are classified into three group according to the magnetic resonance imagings. Patients who have cochlear nerve deficiency may not hear with cochlear amplifications. Auditory brain stem implant (ABI) is most suitable for these cases. In this case report, we presented a 3 years old girls with bilateral totally hearing loss who performed cochlear implantation despite of cochlear nerve deficiency on magnetic resonance imaging (MRI) and computed tomography (CT).

“…Most of the studies considered the indication of ABIs for patients with bilateral severe sensorineural hearing loss caused by issues in the cochlear nerve (aplasia or avulsion after a head trauma with bilateral temporal bone fracture, tumors, like sporadic schwannoma of the cochlear nerve, or bilateral tumors caused by neurofibromatosis type 2 [NF2]) [1,[3][4][5][6][7]8] , severe abnormalities of the cochlea (malformations or ossification) [9] , or auditory neuropathy. [1] It is impossible to estimate the average age of the patients in the studies, since most of them showed results involving patients older than 18 years old.…”

Section: Review Of the Literaturementioning

confidence: 99%

“…Regarding the surgical procedure, 4 patients [13] were fitted with ABIs with the retrolabyrinthine approach, 8 with the suboccipital approach [14] , 49 with the retrosigmoid approach, [10,18,21] and 2 with the translabyrinthine approach [1,3,6,7,9,[13][14][15][22][23][24] . Both patients with bilateral schwannomas and tumors caused by NF2 received the retrosigmoid and transmeatal combined approaches for tumor removal.…”

Section: Review Of the Literaturementioning

confidence: 99%

Auditory Brainstem Implants in Children: Results Based on a Review of the Literature

Monsanto¹,

Bittencourt

Neto³

et al. 2015

The objective of this study is to review the literature regarding Auditory Brainstem Implant (ABI) indications, surgical techniques, activation methods, and post-surgery follow-up in children. A search was performed in the LILACS, MEDLINE, SciELO, and PubMed databases in June 2014, and the key words used in the search were (("auditory brain stem implant" OR "auditory brainstem implants") OR ("auditory" AND "brainstem" AND ("implants" OR "implant")). Forty-two studies that met the criteria described in "Study Selection" were read in full; 24 studies referred to the ABI fitting process in children, and were selected for appraisal. The studies showed 120 children (younger than 18 years old) fitted with ABIs. Evaluation after surgery showed that 112 (93.3%) of the patients improved in their ability to recognize environmental sounds and speech perception. Patients with tumors or those with cochlear or cochlear nerve malformations had good outcomes as well. Two of the children did not achieve any sound perception upon ABI activation. The results obtained in 120 children fitted with an ABI showed that the patients globally improved in their ability to detect sounds and communication skills. The phenomenon could be seen both in patients with tumoral diseases of the inner ear and those with malformations of the cochlea or cochlear nerve, although patients with non-tumoral issues achieved better results than patients with schwannomas. We propose that the Food and Drug Administration (FDA) ABI indications should be extended to patients younger than 12 years old with NT diseases of the cochlea and cochlear nerve.