Extraskeletal chondroma is a rare benign tumor that develops in the soft tissues, and it manifests as a solitary subcutaneous nodule or a slowly growing tumor. It occurs preferentially in the hand and foot area of adults, and the development in the auricle is very rare. We report a rare case of chondroma that developed in the auricle, which were treated with wedge excision and reconstructed using a Burow's triangle, with a review of the literature.
Adult ; Chondroma* ; Foot ; Hand ; Humans

Congenital anomaly of the oval window with an abnormal facial nerve course is an uncommon embryological defect, which is related to the underdevelopment of second branchial arch derivatives. Some treatments for improving hearing levels are available; these include hearing aids, vestibulotomy, neo-oval window formation, and stapes surgeries, including incudostapedotomy and malleostapedotomy. However, surgery for congenital anomalies of the oval window has rarely been described, usually in very small series of patients. We describe two cases of congenital anomalies of the oval window with aberrant facial nerve courses. One was a 40-year-old male diagnosed with unilateral congenital oval window atresia; the other was a 10-year-old male diagnosed with bilateral congenital oval window atresia. We also describe the clinical manifestations and treatment outcomes of malleostapedotomy for congenital anomalies of the oval window with aberrant facial nerve courses.
Adult ; Branchial Region ; Child ; Facial Nerve ; Hearing ; Hearing Aids ; Hearing Loss, Conductive ; Humans ; Male ; Stapes Surgery

Spontaneous cerebrospinal fluid (CSF) otorrhea is defined as CSF otorrhea where there are no identifiable causes including previous trauma, surgery, infection, neoplasm or congenital anomaly. The condition is rare. The origin of CSF leak is commonly a defect in the tegmen of the middle cranial fossa. The pathophysiology of spontaneous CSF otorrhea is unclear. Two theories of the etiology of bony defects of the temporal bone are the congenital bony defect theory and arachnoid granulation theory. The authors experienced a case of a 49-year-old female patient admitted with the complaint of persistent right ear fullness. Computed tomography revealed a large defect of the middle fossa and suspicious CSF otorrhea through the defect of tegmen tympani. Repair was successful with multiple bone chips using the transmastoid approach. The postoperative course was good and there has been no recurrence of the CSF leakage.
Arachnoid ; Cerebrospinal Fluid ; Cerebrospinal Fluid Otorrhea* ; Cranial Fossa, Middle* ; Ear ; Female ; Humans ; Middle Aged ; Recurrence ; Temporal Bone

Achondroplasia is the most common skeletal dysplasia and it combines various complications with normal longevity. Hearing disturbance due to otitis media or an ossicular anomaly is one of the most common complications. Conductive hearing loss is suggested as the most common form of hearing loss. Temporal bone and middle ear structures are distorted in achondroplasia because of rotational change of the skull base. Authors experienced a case of an achondroplastic patient with bilateral hearing disturbance. We faced making a potential mistake during the previous operation but a favorable postoperative result occurred. Our experience could be helpful to other clinicians who face achondroplastic patients.
Achondroplasia* ; Ear Ossicles ; Ear, Middle ; Hearing ; Hearing Loss ; Hearing Loss, Conductive ; Humans ; Longevity ; Ossicular Prosthesis ; Otitis Media ; Skull Base ; Temporal Bone

Newborn hearing screening test is very important in the early diagnosis of childhood hearing loss because it affects language development. Auditory neuropathy is a spectrum disorder characterized by abnormal auditory brainstem response but preserved otoacoustic emission and cochlear microphonics. In general, auditory neuropathy patients have poor word discrimination and variable patterns of pure tone audiometry. We report on a patient with auditory neuropathy diagnosed at 16 months of age and started wearing hearing aids, but showed normal pure tone and speech audiometric findings 3 years later. Close follow-up for patients with auditory neuropathy is recommended.
Audiometry ; Discrimination (Psychology) ; Early Diagnosis ; Evoked Potentials, Auditory, Brain Stem ; Hearing Aids ; Hearing Loss ; Hearing* ; Humans ; Infant, Newborn ; Language Development ; Mass Screening

A 43 year-old female patient suffered the sudden onset of pulsatile tinnitus in the left ear 2 months ago. The tinnitus did not subside spontaneously and remained unchanged. The patient had no history of head trauma or surgery of the head and neck. The character of the tinnitus was pulsatile, and it was synchronous with the heart beat. Audiologic examinations were performed and all of the results were normal. Computed tomography with angiography was performed and evidence of an arterio-venous fistula (AVF) was found. 4-vessel angiography was performed to confirm the dural AVF between the external carotid artery and sigmoid sinus. Embolization of the feeder-vessels was done under a fluoroscope and 70% of the fistula flow was controlled after embolization and the tinnitus totally subsided during the embolization.
Angiography ; Arteriovenous Fistula ; Carotid Artery, External ; Colon, Sigmoid ; Craniocerebral Trauma ; Ear ; Female ; Fistula* ; Head ; Heart ; Humans ; Neck ; Tinnitus*

BACKGROUND AND OBJECTIVES: P1-N1-P2 complex reflecting pre-attentive processing of sound presents several temporally overlapping and spatially distributed neural sources in or near primary auditory cortex. This study investigated cortical evoked responses to the P1-N1-P2 complex to determine the perceptual contributions of the acoustic features. SUBJECTS AND METHODS: Eleven young native-speaking Korean adults with normal hearing participated. The stimuli were three bilabial, three alveolar, and three velar syllables, and each place of articulation had one lax, one tense, and one aspirate syllable as the manner of articulation. RESULTS: The results indicate the cortical responses to the velar syllables significantly differed from the bilabial and alveolar groups at the P1-N1 and N1-P2 interamplitude. However, there is no significant difference in the cortical responses between Korean lax and tense syllables, which is significant for English phonology in terms of voice onset time. Further, the cortical responses to aspirate syllables significantly differed from two other groups in the interamplitude, demonstrating that the /tha/ syllable had the largest response at N1-P2 interamplitude. CONCLUSIONS: Different speech sounds evoked different P1-N1-P2 patterns in the place and the manner of articulation in terms of interamplitude, but not of the latency and interlatency although further studies should be followed.
Acoustics ; Adult ; Auditory Cortex ; Hearing* ; Humans ; Phonetics ; Speech Perception ; Transcutaneous Electric Nerve Stimulation ; Voice

BACKGROUND AND OBJECTIVES: Turner and colleagues introduced a new method that can detect tinnitus in animals. The stimulus is composed of a small background noise that is identical to the pitch of the tinnitus and a large pulse noise that can evoke a startle response. In normal rats, the gap decreases the startle reflex. However, in tinnitus rats, the gap does not decrease the startle reflex. The goal of this study was to optimize the stimulation paradigm so that the prepulse inhibition of N1-P2 amplitude would be maximized in the normal human subjects. SUBJECTS AND METHODS: Seven normal control subjects without tinnitus were recruited. The stimulus was composed of two different sounds: the softer background noise and the louder pulse noise. A 50 msec silent gap was inserted before the pulse noise as the gap condition (G condition) but not in the no-gap condition (N condition). The averaged amplitude of the N1-P2 cortical response was recorded for the G and N conditions. RESULTS: The G/N ratio was the smallest when the gap was 20 msec prior to the pulse noise. The G/N ratio was 84.8+/-16.8% with the Hanning window and 78.5+/-5.9% without the window. The G/N ratio was 91.1+/-24.9%, 78.0+/-5.4%, and 79.0+/-18.1% when the intensity of the background noise was 10, 20, and 32 dB SL, respectively. When the intensity of the background noise was 20 and 32 dB SL, the N1-P2 amplitude of the G condition was significantly smaller than that of the N condition. CONCLUSIONS: The optimal stimulus should be composed of the 1 kHz pulse noise without Hanning window. The intensity of the background noise should be 20 dB HL and the location of the gap should be 20 msec prior to the pulse noise. It seems that with these optimized parameters we could expect a 78.0% inhibition of N1-P2 amplitude in normal subjects without tinnitus.
Animals ; Humans* ; Methods ; Noise ; Rats ; Reflex ; Tinnitus

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

BACKGROUND AND OBJECTIVES: The purpose of this study was to determine how human neural activity might be changed through auditory short-term training when listening to novel stimuli. SUBJECTS AND METHODS: Among the twenty young normal hearing adult listeners who participated, ten were randomly assigned to a training group and ten were assigned to a non-training group as a control. Two synthesized novel stimuli were used: /su/ and /integralu/. Both stimuli similarly sounded like /su/, but had two different onset transition frequencies and fricative pole frequencies. In the experiment, behavioral identification test (i.e., /su/ vs. /integralu/) and the mismatch negativity (MMN) were measured before and after training for the training group. To gauge the training effect, the listeners in the training group were taught by discrimination and identification of two novel stimuli for about 20 minutes. RESULTS: The results showed that scores for the behavioral test increased significantly after auditory short-term training. Also, onset latency, duration, and area of the MMN were significantly changed when elicited by the training stimuli. CONCLUSIONS: These findings indicated that auditory short-term training could change human neural activity, suggesting future clinical applications for auditory training.
Adult ; Discrimination (Psychology) ; Hearing ; Humans