The syndrome with knuckle pads, leukonychia, deafness and palmoplantar hyperkeratosis have been presented by several previous reports since the Bart and Pumphrey's report. This syndrome is that inheritance pattern is autosomal dominant trait. We report a case of a family with hereditary progressive palmoplantar hyperkeratosis, which is invariably associated with knuckle pads and a progressive mixed hearing loss. We think that these traits may not be inherited as an autosomal dominant trait because affected members of the family are all female but may be possibly autosomal dominant. The difference with other previous reports is that all members of the family don't have leukonychia. Therefore, we suggest that this is another variant case of this syndrome.
Deafness* ; Female ; Hearing Loss ; Hearing Loss, Mixed Conductive-Sensorineural ; Humans ; Inheritance Patterns

BACKGROUND AND OBJECTIVES: Conductive hearing loss is common among children and adults. This study aims at comparing the results of conductive hearing loss in summer and autumn. SUBJECTS AND METHODS: Puretone audiometry and tympanometry tests were done for all patients who referred to the Iranian-based audiology center of Imam Khomeini Hospital in Ahvaz. Data on the patients with conductive or mixed hearing loss were analyzed. The impacts of season, age, and etiology of the disease were analyzed on the patients who visited the audiology clinic due to the conductive hearing loss in summer and autumn. RESULTS: One hundred and fifty nine patients in summer and 123 patients in autumn had conductive or mixed hearing loss. Their age ranged from four to 82 years, with the average age of 35. The percentage of the patients, with acute otitis media and chronic otitis media (COM), who visited this clinic, was significantly higher than those with middle ear problems. COM and mastoid surgeries rate was higher in summer than autumn among adults. CONCLUSIONS: There is no relationship between season and middle ear diseases between children and juveniles, but COM and mastoid problems are more common in summer among adults visiting this clinic. Most of the patients had mild conductive hearing loss and bilateral middle ear impairments.
Acoustic Impedance Tests ; Adult ; Audiology ; Audiometry ; Child ; Ear ; Ear, Middle ; Hearing Disorders ; Hearing Loss ; Hearing Loss, Conductive ; Hearing Loss, Mixed Conductive-Sensorineural* ; Humans ; Mastoid ; Otitis Media ; Seasons

Various bone conduction hearing aids have been widely used to improve hearing fuction for the last 30 years. Sophono(R), a newly introduced device without percutaneous abutment, improved disadvantages of previously used bone conduction hearing aids. Recently, we experienced a case of Sophono(R) implantation in a patient presenting with mixed hearing loss followed by tumor surgery. The patient gained air conduction threshold of 60 dB HL compared to preoperative pure tone audiogram and a speech discrimination test in noisy environment showed improved results. Furthermore, the patient has had no cutaneous complications since implantation. The results of this case demonstrate the feasibility of implanting Sophono(R) in patients similar to this case. Considering its advantages of MRI compatibility and non-invasive surgery, as well as esthetical benefit, Sophono(R) implantation could be a preferable option for hearing rehabilitation.
Bone Conduction ; Hearing Loss, Mixed Conductive-Sensorineural ; Hearing Loss, Unilateral* ; Hearing* ; Humans ; Magnetic Resonance Imaging ; Meningioma* ; Rehabilitation* ; Speech Discrimination Tests

The Vibrant Soundbridge (VSB) middle ear implant is an alternative rehabilitation option for sensorineural, conductive, and mixed hearing loss. The mechanism of VSB implantation is direct-drive stimulation of the inner ear through a floating mass transducer placed on the ossicular chain. We report our experience of VSB incus vibroplasty, which were successfully performed in two cases without complications and resulted in good functional gain and improve-ment of speech discrimination.
Ear, Inner ; Ear, Middle ; Hearing Loss, Mixed Conductive-Sensorineural ; Incus ; Ossicular Prosthesis ; Speech Perception ; Transducers

OBJECTIVES: New signal processing technologies have recently become available for Baha(R) sound processors. These technologies have led to an increase in power and to the implementation of directional microphones. For any new technology, it is important to evaluate the degree of benefit under different listening situations. METHODS: Twenty wearers of the Baha osseointegrated hearing system participated in the investigation. The control sound processor was the Baha Intenso and the test sound processor was the Cochlear(TM) Baha(R) BP110power. Performance was evaluated in terms of free-field audibility with narrow band noise stimuli. Speech recognition of monosyllabic phonetically balanced (PB) words in quiet was performed at three intensity settings (50, 65, and 80 dB sound pressure level [SPL]) with materials presented at 0 degrees azimuth. Speech recognition of sentences in noise using the Hearing in Noise Test (HINT) in an adaptive framework was performed with speech from 0 degrees and noise held constant at 65 dB SPL from 180 degrees. Testing was performed in both the omni and directional microphone settings. Loudness growth was assessed in randomly presented 10 dB steps between 30 and 90 dB SPL to narrow band noise stimuli at 500 Hz and 3,000 Hz. RESULTS: The test sound processor had significantly improved high frequency audibility (3,000-8,000 Hz). Speech recognition of PB words in quiet at three different intensity levels (50, 65, and 80 dB SPL) indicated a significant difference in terms of level (P<0.0001) but not for sound processor type (P>0.05). Speech recognition of sentences in noise demonstrated a 2.5 dB signal-to-noise ratio (SNR) improvement in performance for the test sound processor. The directional microphone provided an additional 2.3 dB SNR improvement in speech recognition (P<0.0001). Loudness growth functions demonstrated similar performance, indicating that both sound processors had sufficient headroom and amplification for the required hearing loss. CONCLUSION: The test sound processor demonstrated significant improvements in the most challenging listening situation (speech recognition in noise). The implementation of a directional microphone demonstrated a further potential improvement in hearing performance. Both the control and test sound processors demonstrated good performance in terms of audibility, word recognition in quiet and loudness growth.
Bone Conduction ; Hearing ; Hearing Aids ; Hearing Loss, Mixed Conductive-Sensorineural ; Humans ; Noise ; Osseointegration ; Sensory Aids ; Signal-To-Noise Ratio

It is challenging to achieve sufficient hearing gain in patients with mixed hearing loss. In chronic middle ear diseases, conventional passive reconstructive surgeries often result in suboptimal hearing gain and additional hearing aids may have limitations due to insufficient sound amplification, occlusion effect, acoustic feedback, and skin irritation. Middle ear implantation (MEI) using Vibrant Soundbridge (VSB) is another option for auditory rehabilitation in mixed hearing loss as well as sensorineural hearing loss. The floating mass transducer of VSB can be placed on various middle ear structures either directly or using different types of couplers in order to deliver vibratory mechanical energy to the cochlea. We report a patient who presented with bilateral mixed hearing loss due to chronic otitis media and had limitations using conventional hearing aids in the worse hearing ear. The patient was successfully treated with MEI using the Bell coupler together with middle ear surgery in a single step.
Acoustics ; Cochlea ; Ear ; Ear, Middle* ; Hearing ; Hearing Aids ; Hearing Loss, Mixed Conductive-Sensorineural* ; Hearing Loss, Sensorineural ; Humans ; Ossicular Prosthesis* ; Otitis Media ; Rehabilitation ; Skin ; Transducers

BACKGROUND AND OBJECTIVES: This study aims to evaluate that usefulness of the endoscopic ear surgery (EES) through the systematic review. SUBJECTS AND METHOD: We searched literatures in literature databases (MEDLINE, EMBASE, Cochrane Library, etc.). Inclusion criteria is 1) studies of patients with chronic otitis media, otitis media with effusion, cholesteatoma, conductive hearing loss, mixed hearing loss etc. 2) studies in which a transcanal endoscopic surgery was performed; and 3) studies in which one or more of the appropriate medical outcomes have been reported. We excluded that 1) non-human studies and pre-clinical studies; 2) non-original articles, for example, non-systematic reviews; editorial, letter and opinion pieces; 3) research not published in Korean and English; and 4) grey literature. Finally, 65 articles were selected and those results were analyzed. RESULTS: The safety of the EES was reported in 61 articles. Some studies reported damaged facial nerve or perilymph gusher but these are the complications that can arise due to the characteristics of the disease and not due to the EES and other reported complications were of similar or lower level in the intervention group rather than the microscopy group. The effectiveness of the EES was reported in 23 articles. The EES tended to show improved effects in terms of graft uptake status, cholesteatoma removal, and hearing improvement although effective outcomes of most studies reported no significant difference between EES and microscopic ear surgery. CONCLUSION: EES is a safe and effective technique and as it is less invasive than the microscopic ear surgery.
Cholesteatoma ; Ear ; Endoscopes ; Facial Nerve ; Hearing ; Hearing Loss, Conductive ; Hearing Loss, Mixed Conductive-Sensorineural ; Humans ; Methods ; Microscopy ; Otitis Media ; Otitis Media with Effusion ; Otologic Surgical Procedures ; Perilymph ; Transplants

BACKGROUND AND OBJECTIVES: We evaluated the clinical characteristics of hearing impairment in patients who suffered a blunt head trauma without any organic problems, including temporal bone fracture or intracranial hemorrhage. SUBJECTS AND METHOD: This retrospective study examined 42 patients presenting with hearing impairment after blunt head trauma within five recent years. This study included only patients without temporal bone fracture or intracranial hemorrhage. RESULTS: Most patients (90.5%) complained of associated auditory symptoms including tinnitus, dizziness, earfullness and otalgia as well as headache. In 38 patients (90.5%), the symptom was developed on the injured day. Fifteen ears of 13 patients showed sensorineural hearing loss, 2 ears of 2 patients conductive hearing loss, and 10 ears of 8 patients mixed hearing loss. Twenty-four ears of 22 patients showed sensorineural hearing loss only above 4 kHz, 8 ears of 7 patients mild hearing loss, 10 ears of 8 patients moderate hearing loss, 3 ears of 3 patients had a moderately-severe hearing loss, and 6 ears of 6 patients had a profound hearing loss. All cases (24 ears of 22 patients) who had a normal four-tone average complained many otologic symptoms other than a hearing loss. CONCLUSION: Blunt head injury is one of the most common causes of the neurologic disorders. It is important to perform thorough assessment of auditory symptoms as soon as possible. Otologic consultation should be sought in all cases for appropriate management.
Craniocerebral Trauma* ; Dizziness ; Ear ; Earache ; Head Injuries, Closed ; Head* ; Headache ; Hearing Loss* ; Hearing Loss, Conductive ; Hearing Loss, Mixed Conductive-Sensorineural ; Hearing Loss, Sensorineural ; Hearing* ; Humans ; Intracranial Hemorrhages ; Nervous System Diseases ; Post-Concussion Syndrome ; Retrospective Studies ; Temporal Bone ; Tinnitus

BACKGROUND AND OBJECTIVES: Many authors(Simmons,1973, McGath et al.,1989) described that a small percentage(1-2 %) of patients initially complaining of vertigo was found to have bilateral vestibular failure (BLVF). This study evaluates the findings of vestibular function test (VFT) including rotating chair test (ROT) and computerized dynamic posturography test (CDP) in the patients with BLVF demonstrated by caloric test and others. Method : 400 vertigo patients were given VFT from June,1994 to October,1995. In the patients who had absent or reduced responses (total eye speed <10 degrees per second) in cold (17 C) water caloric testing or decreased responses in the range of lower fequencies during ROT, we evaluated their VFT results and clinical features. RESULTS: There were 38 cases out of total 400 patients(9.5%). Many patients with BLVF had rotational response magnitudes (at 0.04 Hz and above) that were within normal limits. CBP has shown vestibular dysfunction patterns in 32 patients. Hearing tests were normal in 9 cases and mixed hearing loss in 2 cases. Many patients experienced several episodes of acute vertigo with persistent imbalance; Only 4 patients complained of persistent oscillopsia. Viral infection (vestibuloneuronitis), immune reactions and toxic factors may play a role in the etiology of this rare BLVF. CONCLUSION: Our incidence of 9.5% is much higher than what was reported in the literature. Herb medications and frequent injections of aminoglycosides may be responsible for this higher incidence of BLVF. ROT is useful in detecting and predicting the remaining vestibular function in the BLVF patients.
Aminoglycosides ; Caloric Tests ; Electronystagmography ; Hearing Loss, Mixed Conductive-Sensorineural ; Hearing Tests ; Humans ; Incidence ; Vertigo ; Vestibular Function Tests* ; Water

OBJECTIVES: The problem of noise-induced hearing loss (NIHL) was objectively and systematically investigated by diagnosing three workers who complained of tinnitus and hearing disturbance. METHODS: Atypical hearing loss is diagnosed as a work-related disease by using general medical methods, such as environmental survey, neurological examination, otoscopy, pure-tone audiometry, speech audiometry, otoacoustic emissions (OAE), auditory-evoked potentials, and interview on the history of past disease, family and occupation. RESULTS: Three results were found according to the work-related hearing loss as follows; (1) hearing loss that is caused by exposure to continuous noise of less than 85 dB(A) or impact noise of greater than 135 dB peak, (2) mixed hearing loss that has progressed due to noise with past otitis media, and (3) atypical hearing loss that showed remarkable differences between air and bone conduction due to tinnitus. CONCLUSIONS: The criteria for work-related hearing loss should be carefully investigated by considering exposure to impact noise, the effect of continuous noise on mixed hearing loss patients, and the hearing loss caused by tinnitus.
Audiometry, Pure-Tone ; Audiometry, Speech ; Bone Conduction ; Hearing ; Hearing Loss ; Hearing Loss, Mixed Conductive-Sensorineural ; Hearing Loss, Noise-Induced* ; Humans ; Neurologic Examination ; Noise ; Occupations ; Otitis Media ; Otoscopy ; Tinnitus