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Address for correspondence : Kyoung Ho Park, MD, Department of Otolaryngology-Head and Neck Surgery, College of Medicine, The Catholic University of Korea, 505 Banpo-dong, Seocho-gu, Seoul 137-701, Korea
Tel : +82-2-2258-6213, Fax : +82-2-595-1354, E-mail : khpent@catholic.ac.kr
Introduction
Superior semicircular canal dehiscence syndrome (SSCD) is a syndrome of sound or pressure induced vertigo because of bone dehiscence over the Superior semicircular canal. Semicircular canal dehiscence is rare but well-described condition to be induced noise or straining. With dehiscence of bone separating, superior semicircular canal and middle cranial fossa or superior petrosal sinus creates novel pathways for sound transmission and endolymphatic movement.1)
Patients with SSCD accompany with variety of symptoms. Complaint of vertigo, which is triggered by straining, heavy lifting, or loud sound is usually common in low frequency sound. And vestibular symptoms with pulsatile tinnitus, autophonia, conductive hearing loss or conductive hyperacusis are also prevalent.2)
Multi-dimensional treatment is recommended to encourage patient's self-management on various symptoms of SSCD through surgery, anti-depressant medication, anti-emetics and cognitive behavior therapy.
The presented case is female patient who has been successfully improved in vertigo symptoms and depressant and anxiety symptoms through multidimensional treatment.
Case Report
A 51-year old female patient presented with intermittent vertigo and left-side aural fullness for one month after traffic accident, especially left sided head trauma. Loud sound evoked sensation of vertigo and disequilibrium. Vestibular symptoms were not appeared except loud sound, noise condition. These symptom leads to significant restriction in her activity and participation, because she was musician, and she suffered from cymbal sound.
The tympanic membranes were normal and audiometry showed conductive hearing loss with a maximum air-bone gap of 30 dB at 250 Hz in the left ear (Fig. 1). There was no Tullio phenomenon or Hennebert sign. Valsalva maneuver did not evoke any eye movements or a sensation of oscillopsia. Stapedial reflex was present, and the VEMP threshold was 95 dB in the bilateral ear.
The vestibular testing did not find any gaze nystagmus, or positional and positioning nystagmus under infrared CCD camera observation. Caloric stimulation elicited normal nystagmus reaction bilaterally. The maximum slow phase velocities in the right and left ear to the warm water stimulation were 13°/s, 11°/s. The eye tracking test and optokinetic nystagmus test were also normal. On high-resolution temporal bone CT documents dehiscence of left superior semicircular canal along the floor of the middle cranial fossa (Fig. 2).
We suspected the diagnosis was SSCD. Because she was not incapacitating symptoms, our goal of the treatment was to control symptoms, reduce functional disability and improve her quality of life. She was treated with drug prescriptions, vestibular rehabilitation protocols and psychotherapy, instead of surgical repair of the affected canal. Prior to treatment, we explored her dizziness attack with the use of behavior analysis. And any factor that triggers, sustains or intensifies the dizziness was analyzed and evaluated. We let her know and understand the disease and symptoms, in combination with avoidance of loud sounds and Valsalva-like maneuvers. Moreover, relaxation techniques were taught to minimize the disorienting effects of vestibular attack. Graded exercises are utilized to train the brain to increase the threshold to vertigo and to promote postural stability. After educating our vestibular rehabilitating protocol, she performed self vestibular rehabilitation about 1 hour everyday for 3 months. In consultation with psychiatrist, she was received psychotherapy every 2 weeks for 3 months. We measured dizziness, anxiety and depression before and after treatment using standardized questionnaires. The Dizziness Handicap Inventory was used to assess the self-perception of handicapping effects caused by dizziness before and after therapy.3) The Beck Depression Inventory Second Edition was also used to assess the existence and severity of symptoms of depression and anxiety. At 3 months after treatment, the frequency of intermittent vertigo has been decreased and the symptoms of anxiety-depression, panic and agoraphobia was released. She needed no more emergency medication for dizziness symptom after being received cognitive therapy for 3 months. DHI was 32 from 78 and BDI was 15 from 37.
Discussion
SSCD causes vestibular and auditory symptoms and signs as a consequence of the third mobile window in the inner ear created by the dehiscence. The syndrome typically evolves during adulthood and is usually triggered by a sudden change in middle ear or intracranial pressure (excessive straining, head trauma etc). The diagnosis of SSCD was established on both the presence of bone defect overlying superior semicircular canal which was demonstrated using high resolution temporal bone CT scan, and the presence of associated vestibular and auditory symptoms and signs.4)
The management of SSCD involves conservative and surgical approaches. In case of disabling vestibular symptoms of SSCD, surgical intervention may be considered as a valid therapeutic option. The surgical approach options include middle cranial fossa and transmastoid. Even though surgical approach is successful in resolving symptoms and signs, there are considerable complication rates. The major complications associated with surgical approach are sensorineural hearing loss, conductive hearing loss, vestibular hypofunction, dural tear. The minor complications are hematoma, cellulitis, tinnitus.5,6)
Medical treatment is also considered for treatment, even though no medical treatment has been proven fully effective for SSCD. Vestibular rehabilitation is a physical therapy program commonly included in the treatment of patients with peripheral vestibular disorders to train the brain to compensate for defective or abnormal vestibular inputs, to improve the role of alternative visual or proprioceptive inputs.7)
Cognitive behavioral therapy uses relaxation, cognitive restructuring of the thoughts and exposure to exacerbating situations in order to promote habituation and may benefit vestibular disability patients, as may the treatment of associated psychological conditions.
The effect of a combined program vestibular rehabilitation therapy and anxiety management on vertigo symptoms and psychological compensation and emotional distress is significantly decreased vertigo symptoms, anxiety, and emotional distress.11) Psychological disturbance is more among patients with balance disorders than in patients with other disorder. One study has shown that nearly 50% of people complaining of dizziness also report some psychological problems, with more than 25% of dizzy patients presenting symptoms of panic and agoraphobia, and a co-morbid prevalence of dizziness and anxiety of 11%.8,9,10)
Pharmacotherapy and rehabilitation program can be used to reduce the vertigo
patients' functional disability and perceived handicap. However these treatments have limitation from a psychiatric point of view. A lack of social support, a high burden of suffering and moderate to severe impairment of self-experience were associated with the development of a panic disorder after an episode of an acute vestibular disorder.
Dizziness often is accompanied by many types of psychiatric disorder, including depression somatization, and anxiety disorder, and the patient was also very stressful condition.12) We considered not only her vestibular distress but also emotional situation. The first attempt was surgical approach, but she refuse to be operated because she was musician and she had to preserve her hearing ability. The second attempt was conservative care which was included pharmacotherapy, vestibular rehabilitation therapy. She was received psychotherapy every 2 weeks for 3 months and she took daily vestibular rehabilitation exercise by herself for 3 months. And this multidisciplinary treatment produced good result with her faithfulness. The emotional situation is one of the most important prognostic factor, every dizziness patient has to be considered their psychiatric status. And if there is any evidence of psychiatric problem, consultation with psychiatrist could be helpful.
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