TL;DR: The anatomy and physiology of the Auditory Nerve and Ascending Auditory Pathways and the Causes of Inner-Ear Disorders, as well as management of patients with Nonorganic Hearing Loss, are reviewed.
Abstract: I. ELEMENTS OF AUDIOLOGY. 1. The Profession of Audiology. The Evolution of Audiology. Prevalence and Impact of Hearing Loss. Audiology Specialties. Employment Settings. Professional Societies. 2. The Human Ear and Simple Tests of Hearing. Anatomy and Physiology of the Ear. Pathways of Sound. Types of Hearing Loss. Hearing Tests. Tuning Fork Tests. 3. Sound and Its Measurements. Sound. Waves. Vibrations. Frequency. Resonance. Sound Velocity. Wavelength. Phase. Complex Sounds. Intensity. The Decibel. Environmental Sounds. Psychoacoustics. Impedance. Sound Measurement. II. HEARING ASSESSMENT. 4. Pure-Tone Audiometry. The Pure-Tone Audiometer. Test Environment. The Patient's Role in Manual Pure-Tone Audiometry. The Clinician's Role in Manual Pure-Tone Audiometry. Air-Conduction Audiometry. Bone-Conduction Audiometry. Audiogram Interpretation. Masking. The Audiometric Weber Test. Automatic Audiometry. Computerized Audiometry. 5. Speech Audiometry. The Diagnostic Audiometer. Test Environment. The Patient's Role in Speech Audiometry. The Clinician's Role in Speech Audiometry. Speech-Threshold Testing. Masking for SRT. Bone-Conduction SRT. Most Comfortable Loudness Level. Uncomfortable Loudness Level. Range of Comfortable Loudness. Speech-Recognition Testing. Computerized Speech Audiometry. 6. Objective Tests of the Auditory System. Acoustic Immittance. Acoustic Reflexes. Otoacoustic Emissions (OAE). Auditory Evoked Potentials. 7. Behavioral Tests for Site of Lesion. Loudness Recruitment. The Short Increment Sensitivity Index. Tone Decay. Bekesky Audiometry. 8. Hearing Tests for Children. Auditory Responses. Identifying Hearing Loss in Infants under Three Months of Age. Objective Testing in Routine Pediatric Hearing Evaluation. Behavioral Testing of Children from Birth to Approximately Two Years of Age. Behavioral Testing of Children Approximately Two to Five Years of Age. Language Disorders. Auditory Processing Disorders. Psychological Disorders. Developmental Disabilities. Identifying Hearing Loss in the Schools. Nonorganic Hearing Loss in Children. III. HEARING DISORDERS. 9. The Outer Ear. Anatomy and Physiology of the Outer Ear. Development of the Outer Ear. Hearing Loss and the Outer Ear. Disorders of the Outer Ear and Their Treatments. 10. The Middle Ear. Anatomy and Physiology of the Middle Ear. Development of the Middle Ear. Hearing Loss and the Middle Ear. Disorders of the Middle Ear and Their Treatments. Other Causes of Middle-Ear Hearing Loss. 11. The Inner Ear. Anatomy and Physiology of the Inner Ear. Development of the Inner Ear. Hearing Loss and the Inner Ear. Causes of Inner-Ear Disorders. 12. The Auditory Nerve and Central Auditory Pathways. Anatomy and physiology of the Auditory Nerve and Ascending Auditory Pathways. The Descending Auditory Pathways. Development of the Auditory Nerve and Central Auditory Nervous System. Summary of the Auditory Pathways. Hearing Loss and the Auditory Nerve and Central Auditory Pathways. Disorders of the Auditory Nerve. Disorders of the Cochlear Nuclei. Disorders of the Higher Auditory Disorders. Tests for Auditory Processing Disorders. Diagnostic Limitations. Therapeutic Management. 13. Nonorganic Hearing Loss. Terminology. Patients with Nonorganic Hearing Loss. Tests for Nonorganic Hearing Loss. Management of Patients with Nonorganic Hearing Loss. IV. MANAGEMENT OF HEARING LOSS. 14. Amplification/Sensory Systems. Hearing Aid Development. Hearing Aid Circuit Overview. Characteristics of Hearing Aids. Binaural Amplification. Types of Hearing Aids. Selecting Hearing-Aid Candidates. Dispensing Hearing Aids. Verifying Hearing Aid Performance. Hearing Assistance Technologies. 15. Audiological Treatment. Patient Histories. Referral to Other Specialists. Audiological Counseling. Management of Adult Hearing Impairment. Management of Childhood Hearing Impairment. The Deaf Community. Management of Tinnitus. Hyperacusis. Vestibular (Re)habilitation. Multicultural Consideration. Glossary.
TL;DR: A 60-year-old white woman, referred with a "tumor deep in the right ear," had been diagnosed one week previously as having a neurilemmoma of the facial nerve on the basis of a surgical biopsy performed by the referring physician.
Abstract: NEURILEMMOEMA of the facial nerve within the temporal bone is rare with a wide range of clinical symptomatology. Three diverse cases have been encountered by the senior author (G.E.S.), and one additional case by Valvassori. Report of Cases CASE 1.—History.—A 60-year-old white woman, referred with a "tumor deep in the right ear," had been diagnosed one week previously as having a neurilemmoma of the facial nerve on the basis of a surgical biopsy performed by the referring physician. Approximately eight months earlier the patient had consulted the referring physician because of "buzzing" and a pounding tinnitus in her right ear, with a slight hearing loss. At this time the Weber test lateralized to her right ear, and the Rinne test was negative on the right at 256 and 512 cycles per second (cps). She had also noted a slight facial asymmetry with decreased movement on the right, which had slowly progressed.
TL;DR: The skull vibration-induced nystagmus test is a robust, nonintrusive and easy to perform test that is preferable in elderly patients with vascular disease or arthritis of the neck to the head-shaking-test or head-impulse-test.
TL;DR: The Weber test was found to be most sensitive and reliable with the tuning fork stem placed on the upper incisors, and the Rinne tests showed a transition point from Rinne positive to negative at 19 dB when performed using the normal loudness comparison technique and at 24 dB with a threshold comparison technique.
Abstract: The Weber, Rinne and Bing tests were examined in normally hearing and hearing impaired subjects, using different techniques. The Weber test was found to be most sensitive and reliable with the tuning fork stem placed on the upper incisors. The Rinne tests showed a transition point from Rinne positive to negative at 19 dB when performed using the normal loudness comparison technique and at 24 dB with a threshold comparison technique. The Bing test with the tuning fork placed either on the mastoid or the vertex showed a transition point from Bing positive to negative at approximately 9 dB air-bone gap.
TL;DR: The ‘sixteen and a half syndrome’ is described, which is the ‘‘one and ahalf’’ syndrome with an additional 7th and 8th cranial nerve (vestibulo-cochlear nerve) palsy with additional ipsilateral hearing loss.
Abstract: Two neuro-ophthalmological syndromes due to paramedian pontine infarction have previously been described. The ‘‘one and a half’’ syndrome was first reported and named by Miller Fisher [1], and consists of horizontal gaze palsy combined with an ipsilateral inter-nuclear ophthalmoplegia (INO) resulting in loss of all horizontal eye movements except abduction of the contralateral eye. The causative lesion is usually within the ipsilateral pontine tegmentum and is due to involvement of the abducens nucleus plus the medial longitudinal fasciculus (MLF), or alternatively to involvement of the MLF plus the paramedian pontine reticular formation (PPRF). These structures lie in close proximity to the nucleus and intra-axial fasiculus of the facial nerve. Consequently, ipsilateral fascicular lower motor neurone facial nerve palsy may accompany the ‘‘one-and-a-half’’ syndrome. This situation was named the ‘‘eight and a half syndrome’’ by Eggenberger [2], who reported 3 cases of this syndrome that had occured secondary to ischaemic stroke. In continuation of this concept, we here describe the ‘‘sixteen and a half syndrome’’, which is the ‘‘one and a half’’ syndrome with an additional 7th and 8th cranial nerve (vestibulo-cochlear nerve) palsy. A 78-year-old right-handed man presented with sudden onset of incomplete right upper limb paresis, horizontal diplopia and ataxia. This was shortly followed by complete right lower limb paresis. There was associated nausea and vomiting. Examination revealed a left-sided lower motor neurone facial nerve palsy and dysarthria with loss of conjugate gaze to the left and loss of adduction of the left eye. Abduction of the right eye was accompanied by horizontal nystagmus. Convergence was spared. The patient also complained of new-onset left-sided hearing loss. The patient and his family were certain that his hearing before this event had been normal. Magnetic resonance imaging (MRI) with diffusion-weighted sequences revealed an area of restricted diffusion consistent with an acute infarct. The lesion extended beyond and lateral to the paramedian pontine territory supplied by the anteromedial group of pontine perforating arteries, and dorsally to the fourth ventricle and the region of the facial colliculus (Fig. 1a, b). Pure tone audiometry showed no detectable hearing in the left ear. Brainstem auditory evoked potential testing showed no reproducible wave I, wave III or wave V after left ear stimulation. Waves I, III and V from right ear stimulation were of low amplitude with normal absolute and interwave latencies. Eight months later, the Weber test lateralized to the right ear, and the Rinne test revealed absent air-conducted and bone-conducted sound perception in the left ear. Our patient had an ‘‘eight and a half’’ syndrome plus additional ipsilateral hearing loss. We name this clinical entity the ‘sixteen and a half syndrome’. Hearing loss has not previously been described in association with either ‘‘one and a half’’ or ‘‘eight and a half’’ syndrome. A clinical series of 20 cases of ‘‘one and a half’’ syndrome noted G. Cummins R. Dunne K. O’Rourke (&) T. Lynch Dublin Neurological Institute, Mater Misericordiae University Hospital, 57 Eccles St., Dublin 7, Ireland e-mail: killian.orourke@gmail.com