BACKGROUND AND OBJECTIVES: Unilateral auditory dysfunction such as tinnitus and hearing loss could be a warning sign of a retrocochlear lesion. Auditory brainstem response (ABR) and internal auditory canal magnetic resonance image (MRI) are suggested as novel diagnostic tools for retrocochlear lesions. However, the high cost of MRI and the low sensitivity of the ABR test could be an obstacle when assessing patients with unilateral ear symptoms. The purpose of this study was to introduce the clinical usefulness of three-dimensional fast imaging employing steady-state acquisition (3D-FIESTA) MRI in patients with unilateral ear symptoms. SUBJECTS AND METHODS: Two hundred and fifty-three patients with unilateral tinnitus or unilateral hearing loss who underwent 3D-FIESTA temporal bone MRI as a screening test were enrolled. We reviewed the abnormal findings in the 3D-FIESTA images and ear symptoms using the medical records. RESULTS: In patients with unilateral ear symptoms, 51.0% of the patients had tinnitus and 32.8% patients were assessed to have sudden sensory neural hearing loss. With 3D-FIESTA imaging, twelve patients were diagnosed with acoustic neuroma, four with enlarged vestibular aqueduct syndrome, and two with posterior inferior cerebellar artery aneurysm. Inner ear anomalies and vestibulocochlear nerve aplasia could be diagnosed with 3D-FIESTA imaging. CONCLUSIONS: 3D-FIESTA imaging is a highly sensitive method for the diagnosis of cochlear or retrocochlear lesions. 3D-FIESTA imaging is a useful screening tool for patients with unilateral ear symptoms.
Aneurysm ; Arteries ; Diagnosis ; Ear ; Ear, Inner* ; Evoked Potentials, Auditory, Brain Stem ; Hearing Loss ; Hearing Loss, Unilateral ; Humans ; Magnetic Resonance Imaging ; Mass Screening ; Medical Records ; Methods ; Neuroma, Acoustic ; Retrocochlear Diseases ; Temporal Bone ; Tinnitus ; Vestibular Aqueduct ; Vestibulocochlear Nerve

Child ; Cochlear Implantation ; Cochlear Implants ; Evoked Potentials, Auditory, Brain Stem ; Hearing Loss, Central ; physiopathology ; Humans ; Vestibulocochlear Nerve Diseases ; physiopathology

OBJECTIVE: The goal was to know more about the characteristic of auditory neuropathy spectrum disorder (ANSD) for high risk infants. METHOD: The newborn hearing screening was performed with automatic auditory brainstem response (AABR) and transient evoked otoacoustic emission(TEOAE) for the infants in the NICUs from August 2007 to January 2011. After subsequent rescreening, children with AABR test referred were perform hearing test set including high frequency (1000 Hz) tympanometry, ABR, DPOAE and/or Cochlear Microphonics (CMs) in 3 months old. Only infants demonstrated severely abnormal ABRs along with preserved DPOAEs and/or CMs were scheduled for re-examination in 6-8 months old and Behavior audiometry in 8-12 months old. RESULT: Eighteen infants (14 cases were bilateral and 4 cases were unilateral) considered as suffering from AN in 3 months old. All of them showed ABR thresholds > or = 80 dB nHL or absent at maximum test intensity. Follow-up examination revealed 9 cases (18 ears) with restoration of ABR to normal or a lower ABR thresholds and a resolution of ANSI) in 11 out of 18 infants retested in 6-8 months old. CMs were present in all ears but DPOAE were not present in 4 ears with middle ear pathology in 3 months test. Behavioral hearing of 10 cases ranged from mild (n = 2), moderate(n = 4) to severe and profound loss (n = 4). CONCLUSION ANSD in high risk neonates could show the temporary character. It was too difficult to forecast the prognosis, they would be to follow up to at least 3 years old for newborn ANSD.
Child, Preschool ; Evoked Potentials, Auditory, Brain Stem ; Follow-Up Studies ; Hearing Loss, Central ; diagnosis ; physiopathology ; Humans ; Infant ; Infant, Newborn ; Vestibulocochlear Nerve Diseases ; diagnosis ; physiopathology

Neurofibromatosis type II (NF2) is a genetic disease and is transmitted as an autosomal dominant trait. NF2 is characterized by bilateral acoustic neuroma with multiple tumors of central nervous system. Progressive hearing loss is a significant problem in patients with NF2, and hearing preservation is an important factor for choice of treatment plan, especially in a NF2 patient with only one hearing ear. We report an experience of preservation of hearing in a NF2 patient with only one hearing ear through decompression of the internal auditory canal via middle cranial fossa approach.
Central Nervous System ; Cranial Fossa, Middle ; Decompression* ; Ear* ; Hearing Loss ; Hearing* ; Humans ; Neurofibromatoses* ; Neurofibromatosis 2* ; Neuroma, Acoustic

OBJECTIVETo explore the electrophysiological results and rehabilitation outcome of two prelingually deafened pediatric cochlear implant patients with auditory neuropathy.

METHODSPreoperative audiological evaluation, intra-postoperative electrically evoked auditory brainstem response (EABR) and neural response telemetry (NRT) record for the two cases were conducted in Beijing Tongren Hospital. A one year follow-up was performed. Data collected before and at 6,12-month intervals after implantation were compared with that from control pediatric cochlear implant patients matched for the same duration of implant use as this two cases.

RESULTSThe two children implanted had not had any postoperative medical or cochlear implant device complications. Intraoperative EABR and NRT were elicited in case 1 with unrepeatable waveforms. After 12 months of training, Case 1 had shown significant improvements in sound detection, speech perception abilities and communication skills, which was better than the control group, and the electrophysiological results became normal. Case 2 had also benefited from cochlear implantation, even though no recognizable NRT was found until he returned 12 month after the operation. CONTUSIONS: The desynchronization of auditory path had been changed after the electrical stimulation ongoing 12 months for children with auditory neuropathy. The two children had not had any complications postoperatively, and each child had shown improved listening and communication skills. Cochlear implantation could help patients with auditory neuropathy to improve their communication skill and go back to the main stream.

Child ; Cochlear Implantation ; Cochlear Implants ; Hearing Loss, Sensorineural ; surgery ; Humans ; Infant ; Male ; Retrocochlear Diseases ; surgery ; Treatment Outcome

Hearing Loss, Central ; genetics ; Humans ; Vestibulocochlear Nerve Diseases

Auditory neuropathy is a term used to describe abnormal auditory brain stem response (ABR) in the presence of preserved cochlear outer hair cell functions which can be measured by otoacoustic emissions (OAE). We report a case of auditory neuropathy accompanying unilateral vestibular hypofunction and benign paroxysmal positional vertigo. The patient was a 50-year-old man who had experienced hearing loss and tinnitus which started two weeks ago. He had taken several medicines for the last few months due to his lung cancer and tuberculosis. ABR and OAE were checked and the results were compatible with auditory neuropathy. To evaluate his vestibular function, video nystagmography, rotatory chair and oculomotor test were checked. The results were compatible with left unilateral vestibular loss and left lateral canal cupulolithiasis. But the patient experienced nearly no vertigo during his daily life. As presented in this case, most of the auditory neuropathy patients do not complain of vertigo. This is probably due to long term central compensation or maybe due to the decreased nerve conduction of the vertiginous sensation. Vestibular evaluation may be crucial in order to detect masked vestibular dysfunction and to protect these patients from imbalance accidents.
Compensation and Redress ; Evoked Potentials, Auditory, Brain Stem ; Hair ; Hearing Loss ; Hearing Loss, Central ; Humans ; Lung Neoplasms ; Masks ; Middle Aged ; Neural Conduction ; Sensation ; Tinnitus ; Tuberculosis ; Vertigo ; Vestibular Neuronitis

A speech discrimination test is a test using a list of 25 phonetically balanced monosyllables. It is often overlooked but significant enough for pure tone audiometry. Many physicians have performed pure tone audiometry but without a speech discrimination test. A 73-year-old woman visited our clinic complaining of sudden bilateral hearing loss. Pure tone audiometry showed only bilateral high frequency loss. However, speech discrimination had decreased markedly. We decided to follow-up after 1 week of Ginexin-F(R) (ginkgo leaf extract) and Nafril(R) (nafronyl oxalate). She felt a gait disturbance within 2 days. Magnetic resonance imaging revealed a left thalamic hemorrhage. After a 1 month hospitalization, the hematoma subsided, and speech discrimination recovered 3 months later. Acute hearing loss due to thalamic hemorrhage that recovered has never been reported. We report the first case of retrocochlear hearing loss that occurred with a thalamic hemorrhage in a patient who recovered.
Aged ; Audiometry ; Female ; Follow-Up Studies ; Gait ; Hearing Loss ; Hearing Loss, Bilateral ; Hematoma ; Hemorrhage* ; Hospitalization ; Humans ; Magnetic Resonance Imaging ; Retrocochlear Diseases* ; Speech Discrimination Tests ; Speech Perception ; Thalamus

OBJECTIVETo explore the clinic characteristics, audiological characteristics and location of lesions in children with retrocochlear auditory nerve impairment which, including auditory neuropathy characterized by severely abnormal results of ABR and normal results of DPOAE.

METHODSBetween 2002 and 2006, eighty-six cases (165 ears) with severely abnormal ABR but normal results of DPOAE were enrolled in the study group. The mean patient age was one year and one-month-old, with a range of 8 days to 7 years. The cases with abnormal conductive function were excluded. The cases with severely abnormal ABR and normal cochlear functions as measured by DPOAE and without abnormal conductive function were selected as the cochlear lesion group. Some same age healthy children without hearing loss were subjected as normal control group. The latency and amplitude of waves I, III and V, the inter peak latency I-III was compared among the three group.

RESULTS(1) Fifty-one cases (59.3%) had a history of hypercholesterolemia during neonatal period, but 40 cases (46.51%) had a severe hypercholesterolemia and 11 cases (12.79%) had mild or moderate hypercholesterolemia. Clinical features common among the population included a history of dyskinesia [n = 40 (46.51%)], hearing and language disorder [n = 10 (11. 63%)]. Thirty-two cases (37.2%) were accompanied by cerebral palsy . (2) Among the 165 ears, absent ABRs to click stimuli presented at 103 dB was in 103 ears, only wave I was developed in 27 ears and only wave V was developed in 19 ears,wave I and III in 13 ears and differentiated wave I and V in 3 ears. (3) When compared to control group, the latency of wave I was prolonged and amplitude of wave I was lower in cases with only wave I developed (t = -6.75 and 2.58, P < 0.05). For for cases with only wave I and III differentiated, the latency and amplitude of wave I was the same but the latency of wave III was prolonged and amplitude of wave III was lower while interpeak latency I-III was prolonged.

CONCLUSIONSAuditory neuropathy which was characterized by severely abnormal ABR was the most common type of retrocochlear auditory nerve impairment. It was mainly due to a disorder of VIII nerve. The pathologies that affect higher levels of the auditory pathway, from the brainstem to the auditory cortex, might be the main sites of lesion in cases with only wave I developed. Superior olivary nucleus where wave III was generated and higher levels of the auditory pathway might be the main sites of lesion in cases with wave I and III differentiated. The low-amplitude wave V was not characteristics of auditory neuropathy. Cerebral cortex, brain stem auditory nucleus and VIII nerve might be damaged successively in cases with retrocochlear auditory nerve impairment induced by hypercholesterolemia.

Auditory Pathways ; physiopathology ; Case-Control Studies ; Child ; Child, Preschool ; Cochlear Nerve ; pathology ; Evoked Potentials, Auditory, Brain Stem ; Humans ; Infant ; Infant, Newborn ; Retrocochlear Diseases ; pathology ; physiopathology

BACKGROUND AND OBJECTIVES: There are many audiological tests to distinguish between the hearing loss patients of cochlear lesion and the retrocochlear lesion. We started this research to compare the efficacy of the audiological tests and to make helpful selections of the auditory hearing tests in diagnosis of the sensorineural hearing loss patients. MATERIALS AND METHOD: Our study was conducted on 198 patients with hearing loss. They received otologic physical examination, neurological examination, and MRI. We evaluated SISI, STAT, ARDT, DPOAE, and ABR data values and analysed their sensitivity and specificity. RESULTS: Sensitivity of SISI was 55% and specificity was 74%. Sensitivity of STAT was 58% and specificity was 67%. Sensitivity of ARDT was 44% and specificity was 71%. Sensitivity of DPOAE was 50% and specificity was 64%. Sensitivity of ABR was 90% and specificity was 74%. CONCLUSION: Among the tests performed above, ABR showed the highest sensitivity and specificity value, which implies it to be the most meaningful screening test in the sensorineural hearing loss patients. But for the patients with severe hearing loss, such that the auditory hearing test results are suspicious in its accuracy, or for the patients who are suspicious of retrocochlear lesions based upon multiple tests, MRI should be performed to make the final diagnosis.
Diagnosis ; Diagnosis, Differential* ; Evoked Potentials, Auditory, Brain Stem ; Hearing Loss ; Hearing Loss, Sensorineural ; Hearing Tests ; Humans ; Magnetic Resonance Imaging ; Mass Screening ; Neurologic Examination ; Physical Examination ; Retrocochlear Diseases* ; Sensitivity and Specificity