Granulomatosis with polyangiitisis [(GPA) or Wegener granulomatosis] is a multi-system disease characterized by granuloma formation and necrotizing vasculitis. GPA classically shows involvement of the respiratory tracts and the renal system. However, locoregional disease is common and may include otologic manifestations. Although otologic involvement can occur during the course of GPA, no report has described facial palsy with sudden sensorineural total deafness with vertigo as the presenting feature of GPA. This case describes a patient with multiorgan involving resistant form of GPA initially presenting with bilateral profound sudden sensorineural hearing loss and left facial paralysis with vertigo. The condition responded well to treatment with rituximab.
Deafness ; Facial Paralysis* ; Granuloma ; Hearing Loss, Bilateral ; Hearing Loss, Sensorineural ; Hearing Loss, Sudden* ; Humans ; Respiratory System ; Rituximab ; Vasculitis ; Vertigo

Biotinidase deficiency is a disorder inherited autosomal recessively showing evidence of hearing loss and optic atrophy in addition to seizures, hypotonia, and ataxia. In the present study, a 2-year-old boy with Biotinidase deficiency is presented in which clinical symptoms have been reported with auditory neuropathy/auditory dyssynchrony (AN/AD). In this case, transient-evoked otoacoustic emissions showed bilaterally normal responses representing normal function of outer hair cells. In contrast, acoustic reflex test showed absent reflexes bilaterally, and visual reinforcement audiometry and auditory brainstem responses indicated severe to profound hearing loss in both ears. These results suggest AN/AD in patients with Biotinidase deficiency.
Ataxia ; Audiometry ; Biotinidase Deficiency* ; Biotinidase* ; Child, Preschool ; Ear ; Evoked Potentials, Auditory, Brain Stem ; Hair ; Hearing Loss ; Humans ; Male ; Muscle Hypotonia ; Optic Atrophy ; Reflex, Abnormal ; Reflex, Acoustic ; Seizures

BACKGROUND AND OBJECTIVES: We compared improvements in hearing thresholds in acute low-tone sensorineural hearing loss (ALHL) patients after two different treatments: steroid alone and steroid and diuretic combined. We analyzed how the duration between the onset of symptoms and the initiation of treatment affected hearing loss improvement and investigated the relation between presence of vertigo in ALHL patients and ALHL progression to Ménière's disease (MD). SUBJECTS AND METHODS: We retrospectively analyzed the medical records of 47 ALHL patients aged 21 to 76 years. Patients received either orally administered steroid alone (n=12) or steroid and diuretic combined (n=35). We compared improvements in the two groups' hearing thresholds at three lower frequencies (125, 250, and 500 Hz) after participants had received one month of each respective treatment. RESULTS: Our two treatments did not show any statistical difference in hearing loss improvement after one month. Forty percent of ALHL patients with vertigo developed MD, which was a significantly higher rate than the 12.5% of ALHL patients without vertigo who developed MD. The shorter duration between the onset of symptoms and the initiation of treatment significantly increased improvement in the sum of lower frequency hearing threshold after one month. CONCLUSIONS: The current study suggests that steroid and diuretic administered together and steroid alone similarly improve the hearing threshold in ALHL patients after one month. We concluded that patients should initiate ALHL treatment as soon as they experience symptoms. ALHL patients should also be notified of their higher risk of developing MD.
Hearing ; Hearing Loss ; Hearing Loss, Sensorineural* ; Humans ; Medical Records ; Meniere Disease ; Retrospective Studies ; Vertigo

BACKGROUND AND OBJECTIVES: Pneumatization of air cells in the mastoid bone is decreased in chronic otitis media (COM). A decrease in the size of the external auditory canal (EAC) is also found frequently in patients with COM, but this has been little studied. We compared the size of affected bony EACs and the contralateral side in patients with single-side COM using high-resolution computed tomography. SUBJECTS AND METHODS: In total, 99 patients with single-side COM were included. Four indicators related to the size of the bony EAC and IAC were measured using high-resolution computed tomography: the axial and coronal lengths of the tympanic membrane, the length of the isthmus, and the area of the bony ear canal. We also compared both internal auditory canals as negative controls. These assessments were made by radiologists who were blinded to the objective of this study. RESULTS: In patients with single-side COM, the axial length of the tympanic membrane was significantly shorter than normal, and the volume of the EAC was also significantly smaller. The length of the isthmus of the EAC was shorter on the affected side, but the difference was not significant. The IAC volume showed no difference between the two sides. CONCLUSIONS: COM affects general temporal bony development, including the bony EAC and mastoid bone. Therefore, whether to correct this should be considered when preparing for COM surgery.
Ear Canal* ; Humans ; Mastoid ; Otitis Media* ; Otitis* ; Tympanic Membrane

BACKGROUND AND OBJECTIVES: The epidermoid cyst is a common benign disease of the skin caused by inflammation of hair cortex follicles and proliferation of epidermal cells within the dermis or superficial subcutaneous tissue. The purpose of this study was to investigate the characteristics of epidermoid cysts of the external auditory canal (EAC) by analyzing the clinical and radiologic features. SUBJECTS AND METHODS: The clinical records were retrospectively reviewed for patients diagnosed with epidermoid cyst of the EAC from March 2004 to December 2013. The epidermoid cysts were diagnosed clinically by endoscopy and microscopy examinations and by temporal bone CT images, and were confirmed by histopathologic examination. Characteristics of epidermoid cysts in bony EAC and cartilaginous EAC were compared. RESULTS: Eight patients had an epidermoid cyst in the bony EAC and nine patients had one in the cartilaginous EAC. Swelling and otalgia were common symptoms, but 47% of cysts were found incidentally. The mean age of patients was 49.6 years (age range, 26-67 years) in the bony EAC cases and 26.1 years (age range, 6-57 years) in the cartilaginous EAC cases. The mean size of the epidermoid cyst was 3.50 mm (size range, 2-7 mm) in the bony EAC cases and 9.55 mm (size range, 2-20 mm) in the cartilaginous EAC cases. CONCLUSIONS: Comparison of epidermoid cysts of the bony EAC and the cartilaginous EAC revealed that epidermoid cysts of the bony EAC is usually found incidentally, arose in older patients and had smaller size.
Dermis ; Ear Canal* ; Earache ; Endoscopy ; Epidermal Cyst* ; Hair ; Humans ; Incidental Findings ; Inflammation ; Microscopy ; Retrospective Studies ; Skin ; Subcutaneous Tissue ; Temporal Bone

BACKGROUND AND OBJECTIVES: A number of etiologies of idiopathic sudden sensorineural hearing loss (ISSNHL) have been proposed, including viral infection, vascular disturbance, and immune-mediated mechanisms. Intralabyrinthine hemorrhage (ILH) as a cause of SSNHL is extremely rare, and there have been no studies defining the characteristics of hearing impairment and prognosis in patients with ISSNHL due to ILH. This study aimed to investigate the difference in impaired hearing patterns and prognosis for hearing recovery between patients with ISSNHL due to ILH confirmed by magnetic resonance imaging (MRI) and sex- and age-matched patients with ISSNHL due to causes other than ILH. SUBJECTS AND METHODS: We compared the results of audiometry and MRI in 12 patients who had ILH on MRI (hemorrhage group) and in 23 sex- and age-matched controls without abnormal findings related to their hearing loss on MRI (non-hemorrhage group). Initial hearing impairment, progression, and recovery of hearing loss were compared between the two groups. RESULTS: A majority of patients (92%) in the hemorrhage group complained of dizziness. Initial hearing impairment was more frequent in the hemorrhage group than in the non-hemorrhage group (94.09±35.9 vs. 66.66±30.1, p-value=0.036). The final recovery threshold in the hemorrhage group was worse (78.19±46.26 vs. 37.17±31.96, p-value=0.014) than that in the non-hemorrhage group. In the hemorrhage group, hearing recovery seemed to occur less often at high frequencies (2,000, 4,000, and 8,000 Hz) than at low frequencies (250, 500, and 1,000 Hz). CONCLUSIONS: The presence of ILH was associated with poor hearing prognosis and the occurrence of vertigo. The abrupt onset of hearing loss associated with vertigo and the presence of hyperresonance on fat-suppressed T1-weighted MRI images of labyrinthic fluid strongly suggests acute intralabyrinthine hemorrhage, and is predictive of considerable hearing impairment and poor prognosis.
Audiometry ; Dizziness ; Ear, Inner ; Hearing ; Hearing Loss ; Hearing Loss, Sensorineural* ; Hemorrhage* ; Humans ; Magnetic Resonance Imaging ; Prognosis* ; Vertigo

BACKGROUND AND OBJECTIVES: P300 has been studied with a variety of stimuli. However, the nature of P300 has not been investigated for deviant stimuli which change its characteristics from standard stimuli after a period of time from onset. SUBJECTS AND METHODS: Nine young adults with normal hearing participated in the study. The P300 was elicited using an oddball paradigm, the probability of standard and deviant stimuli was 80% and 20% respectively. Six stimuli were used to elicit P300, it included two pure-tones (1,000 Hz and 2,000 Hz) and four tone-complexes (tones with frequency changes). Among these stimuli, 1,000 Hz tone served as standard while others served as deviant stimuli. The P300 was recorded in five separate blocks, with one of the deviant stimuli as target in each block. Electroencephalographic was recorded from electrode sites Fz, Cz, C3, C4, and Pz. Latency and amplitude of components of the cortical auditory evoked potentials were measured at Cz. RESULTS: Waveforms obtained in the present study shows that, all the deviant stimuli elicited obligatory P1-N1-P2 for stimulus onset. 2,000 Hz deviant tone elicited P300 at a latency of 300 ms. While, tone-complexes elicited acoustic change complex (ACC) for frequency changes and finally elicited P300 at a latency of 600 ms. In addition, the results showed shorter latency and larger amplitude ACC and P300 for rising tone-complexes compared to falling tone-complexes. CONCLUSIONS: Tone-complexes elicited distinct waveforms compared to 2,000 Hz deviant tone. Rising tone-complexes which had an increase in frequency elicited shorter latency and larger amplitude responses, which could be attributed to perceptual bias for frequency changes.
Acoustics ; Bias (Epidemiology) ; Doppler Effect ; Electrodes ; Evoked Potentials* ; Evoked Potentials, Auditory ; Hearing ; Humans ; Young Adult

BACKGROUND AND OBJECTIVES: The aim of this study was to investigate serum paraoxanase-1 (PON) activity, total oxidant status (TOS), total antioxidant status (TAS), and the oxidative stress index (OSI) in tinnitus; and to compare the results with data from healthy subjects. SUBJECTS AND METHODS: A total of 114 subjects-54 patients with tinnitus and 60 healthy controls were enrolled in this study. Serum PON activity, TOS, TAS, and OSI levels were measured. RESULTS: In the tinnitus group, TAS, and PON were significantly lower than in the control group (p<0.001). However, the TOS, and OSI levels were significantly higher in the tinnitus group than in the control group (p<0.001). CONCLUSIONS: According to the data obtained from the present study, patients with tinnitus were exposed to potent oxidative stress. Oxidative stress may be the key contributing factor to the pathogenesis of tinnitus.
Aryldialkylphosphatase* ; Humans ; Oxidative Stress ; Tinnitus*

BACKGROUND AND OBJECTIVES: Hyperbillirubinemia in infants have been associated with neuronal damage including in the auditory system. Some researchers have suggested that the bilirubin-induced auditory neuronal damages may be temporary and reversible. This study was aimed at investigating the auditory neuropathy and reversibility of auditory abnormalities in hyperbillirubinemic infants. SUBJECTS AND METHODS: The study participants included 41 full term hyperbilirubinemic infants (mean age 39.24 days) with normal birth weight (3,200-3,700 grams) that admitted in hospital for hyperbillirubinemia and 39 normal infants (mean age 35.54 days) without any hyperbillirubinemia or other hearing loss risk factors for ruling out maturational changes. All infants in hyperbilirubinemic group had serum bilirubin level more than 20 milligram per deciliter and undergone one blood exchange transfusion. Hearing evaluation for each infant was conducted twice: the first one after hyperbilirubinemia treatment and before leaving hospital and the second one three months after the first hearing evaluation. Hearing evaluations included transient evoked otoacoustic emission (TEOAE) screening and auditory brainstem response (ABR) threshold tracing. RESULTS: The TEOAE and ABR results of control group and TEOAE results of the hyperbilirubinemic group did not change significantly from the first to the second evaluation. However, the ABR results of the hyperbilirubinemic group improved significantly from the first to the second assessment (p=0.025). CONCLUSIONS: The results suggest that the bilirubin induced auditory neuronal damage can be reversible over time so we suggest that infants with hyperbilirubinemia who fail the first hearing tests should be reevaluated after 3 months of treatment.
Bilirubin ; Birth Weight ; Evoked Potentials, Auditory, Brain Stem* ; Hearing ; Hearing Loss ; Hearing Tests ; Humans ; Hyperbilirubinemia ; Infant* ; Mass Screening ; Neurons ; Risk Factors

The estimated incidence of acute facial paralysis is approximately 30 patients per 100000 populations annually. Facial paralysis is an extremely frightening situation and gives extreme stress to patients because obvious disfiguring face may cause significant functional, aesthetic, and psychological disturbances. For stressful patients with acute facial paralysis, it is very important for clinicians to answer the questions like whether or not their facial function will return to normal, how much of their facial function will be recovered, and how long this is going to take. It is also important for clinicians to treat the psychological aspects by adequately explaining the prognosis, in addition to providing the appropriate medical treatment. For decades, clinicians have used various electrophysiologic tests, including the nerve excitability test, the maximal stimulation test, electroneurography, and electromyography. In particular, electroneurography is the only objective measure that is useful in early stage of acute facial paralysis. In this review article, we first discuss the pathophysiology of injured peripheral nerve. And then, we describe about various electrophysiologic tests and discuss the electroneurography extensively.
Electromyography ; Facial Paralysis* ; Humans ; Incidence ; Peripheral Nerves ; Prognosis