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Representative neurophysiological responses of normal-hearing and impaired auditory-nerve fibers.
Figure 1: Representative neurophysiological responses of normal-hearing and impaired auditory-nerve fibers. Tuning curves (top panel) and period histograms of tone responses (lower panels) from a representative control fiber (left) and noise-exposed fiber (right).
Department of Speech, Language and Hearing Sciences, Purdue University, West Lafayette, Indiana, USA.
Introduction: The aplasia or hypoplasia of the vestibulocochlear nerve is one of the causes of sensorineural and congenital hearing loss.
INTRODUCTIONThe aplasia or hypoplasia of the vestibulocochlear nerve is the causes of sensorineural hearing loss and congenital deafness.
My dad just had his hearing tested and was told that he has a sensorineural hearing loss.  The person testing his hearing explained to him what that meant.
Before we explain what a sensorineural hearing loss (often abbreviated as SNHL) is, we need to explain how you hear. A sensorineural hearing loss occurs when there is damage to the inner ear (cochlea), or to the nerve pathways from the inner ear to the brain. If your Dad has a sensorineural hearing loss that is interfering with his ability to communicate and impacting his quality of life suggest he give us a call. Upward-pointing arrows along the abscissa of tuning curves indicate characteristic frequency. Aplasia or Hypoplasia of the Vestibulocochlear Nerve in a Infant with Sensorineural Hearing Loss. Their diagnosis is important to candidates for cochlear implant, for being a contraindication for such procedure, by this matter they are suitable for the brainstem implant. Although aplasia or hypoplasia of the vestibulocochlear nerve is rare, their diagnoses have been considered important before surgery for candidates to cochlear implant. Objetive: To present a case of sensorineural hearing loss with definite ethiologic diagnosis. It is a contraindication to surgery combined with the narrowing of internal acoustic meatus (1), and for these cases, brainstem implant is the recommended approach.

Period histograms show fluctuation in mean spike rate over two cycles of the ~1.4-kHz tone stimulus. Case Report: A 11-year-old female patient, Caucasian, whose family complained of her being disattention and dyslalia, by apparently presenting floating hearing. The recent advances on imaging exams enable to achieve detailed definition of the anatomical structures of the internal acoustic meatus when precisely contrasting nerve branches and the Cerebrospinal fluid (CSF) (2).CASE REPORTAn 11-year-old girl, Caucasian, whose family complained about her absence of mind and dyslalia (a speech defect caused by malformation of or imperfect distribution of nerves to the organs of articulation).
Masking condition and vector strength of phase locking to tones are specified above each histogram. The audiometry presented lack of hearing in the left ear and moderate sensorineural hearing loss in high frequencies in the right ear.
CT of the Temporal Bone showed aparent atresia or significant stenosis of the left cochlear foramen and normal right cochlear foramen.
The MRI demonstrated that the left eighth cranial nerve had smaller caliber than the right one and cochlear division was not identified. Auditory exam revealed some hearing in the left ear and sensorineural moderate dysacusia in high frequencies in the right ear (picture 1). Discussion: It is important to find etiology in children with sensorineural hearing loss, once procedures might differ when regarding malformation.
CT of the temporal bones showed apparent atresia or expressive reduction on amplitude of the left cochlear foramen, and normal right cochlear foramen (picture 2).The vestibule and semicircular canal are in normal condition as well as the cochlear and vestibular aqueducts. Conclusion: The objective of this presentation comes to the fact of a rare diagnosis of sensorineural hearing loss, that somehow could be concluded as an idiopathic hearing loss and also it reassures the importance of CT and MRI exam for sensorineural hearing loss. At the Nuclear magnetic resonance (NMR) of the cranium, the caliber of the left eighth cranial nerve presents smaller than the right one and cochlear division is not identified (pictures 3 and 4). It is also noticed discreet hypoplasia of the left modiolus, but no identification of cochlear foramen; it is clearly seen the reduction of the transversed diameter of the cochlea with identification of cochlear scales of the second and third spirals.
The human cochlea portion starts developing with the arising of otic plaque in the third week of embryonary life. Otic plaque is transformed into otic vesicle originating the endolymphatic duct, the utricle, the sacule, the semicircular canals and the cochlea.
In the ninth week the chochlea spirals are already formed and neural epithelium begins to appear.

The neuroblasts of the cochlear ganglion disaggregate from the otic epithelium and the fibers of the cellular bodies of such ganglion grow from the outside into the otic epithelium and into the brainstem (3).
The aplasia or hypoplasia of the vestibulocochlear nerve has already been a matter of suspicious in cases of unsuccessful cochlear implant in which there was no response to electric stimulus and in children with a stressed narrowing of the internal acoustic meatus. Eight children were submitted to cochlear implantation presenting an acoustic meatus of 1-2mm diameter.
Three of them did not present sound perception after surgery, and anatomical findings of the narrowing observed from the high-resolution CT would represent aplasia of the nerve (1).
Histopathological studies on temporal bones also suggest that a well-formed cochlea and an apparently normal Corti's organ might coexist in the absence of the spiral ganglion and the cochlear nerve, and even when the internal acoustic meatus has a normal caliber (5).The aplasia or hypoplasia of the vestibulocochlear nerve, as one of the etiologies of sensorineural and congenital hearing loss, can be diagnosed by MNR of of fine cuts (mm) of the internal acoustic meatus. For presenting a social hearing in accordance with the parameters of normality in the right ear, the diagnosis was late in relation to little hearing to the left, and only one specific audiological exam could determine profound loss of hearing.
Epidemiological studies suggest that one third of the causes of hearing loss is hereditary, another third is acquired and the remaining part is idiopathic (7).FINAL COMMENTSThe report of the current clinical case is due to the fact of a diagnosis of a rare entity on sensorineural hearing loss, which could be considered as an idiopathic loss. It also highlights the importance of both CT and MNR for cases of sensorineural hearing loss. Cochlea implant was not recommended in this case, because patient presents a contralateral hearing that enables her to communicate.REFERENCES1. Formation of the cochlea in the chicken embryo: sequence of innervation and localization of basal lamina - associated molecules. PhD in Medicine by Universidade Federal de Sao Paulo (Federal University) - Escola Paulista de Medicina - UNIFESP (Associated Professor of the Department of Especialized Medicine (ENT Discipline) at Centro de Ciencias da Saude da Universidade Federal do Espirito Santo -UFES) - Health Science Center - Federal University.2. Master degree in Otorhinolaryngology and PhD in Medicine by Universidade Federal de Sao Paulo - UNIFESP - Escola Paulista de Medicina (ENT Doctor in Vitoria, ES - Brazil).3. Physician by Universidade Federal do Espirito Santo (UFES) (Resident doctor of the Clinical Otorhinolaryngoly Discipline at Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo - FMUSP).4. Physician by Universidade Federal do Espirito Santo (UFES) (Radiologist at Multiscan, Vitoria, ES).5.

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