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\begin{document}

\title{Bilateral Semicircular Canal Dehiscence Analyzed Using the International
Classification of Functioning, Disability, and Health (ICF) Framework:
Case report.}



\author[1]{Ignacio Cornejo}%
\author[1]{VIJAYA PRAKASH KRISHNAN MUTHAIAH}%
\author[2]{Victor Mercado-Martinez}%
\author[3]{Gustavo Ulloa-Alvarado }%
\author[4]{Carlos Pino-Urrutia}%
\author[5]{Krishnamoorthy Gunasekaran}%
\affil[1]{University at Buffalo School of Public Health and Health Professions}%
\affil[2]{Instituto de Neurorehabilitación y Balance}%
\affil[3]{Universidad Andres Bello}%
\affil[4]{Instituto de Neurorrehabilitación y Balance}%
\affil[5]{Dambi Dollo University}%


\vspace{-1em}



  
  \date{May 24, 2024}


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\textbf{Bilateral Semicircular Canal Dehiscence Analyzed Using the
International Classification of Functioning, Disability, and Health
(ICF) Framework: Case report.}

Ignacio Novoa-Cornejo\textsuperscript{1} , Vijaya Prakash Krishnan
Muthaiah\textsuperscript{1*}, Victor
Mercado-Martinez\textsuperscript{2},Gustavo Ulloa-Alvarado
\textsuperscript{2,3},Carlos Pino-Urrutia\textsuperscript{2} ,
Krishnamoorthy Gunasekaran

\textsuperscript{1}Department of Rehabilitation Sciences, School of
Public Health and Health Professions, State University of New York at
Buffalo, Buffalo, NY, United States.

\textsuperscript{2} Instituto de Neurorrehabilitaci\selectlanguage{ngerman}ón y Balance, Viña
del Mar, Valparaíso, Chile.

\textsuperscript{3} Exercise and Rehabilitation Sciences Institute,
School of Physical Therapy, Faculty of Rehabilitation Sciences,
Universidad Andres Bello, Santiago 7591538, Chile.

\textsuperscript{4}Department of Medical Biochemistry, College of Health
Sciences, Dambi Dollo University, Dambi Dollo Ethiopia.

* Represents equal first authorship.

\textbf{Abstract} A severe case of bilateral superior semicircular
dehiscence was presented in Instituto de Neurorrehabilitación y Balance,
Chile. The patient reports hearing and vestibular problems in certain
situations; a complete analysis is carried out from the clinical history
to neurological laboratory studies and imaging to diagnose bilateral
semicircular canal dehiscence finally. Health condition management is
under the ICF model, which will allow for determining and classifying
the problems and possible interventions for this interesting clinical
case.

\textbf{Keywords} Bilateral semicircular canal dehiscence, ICF
framework, vestibular disorder.

\textbf{Introduction}

Bilateral semicircular canal dehiscence (B-SCD) is a unique condition
characterized by abnormal openings or thinning of the bony structures in
the inner ear's semicircular canals\textsuperscript{1}. It can manifest
with various auditory and vestibular symptoms, significantly impacting
an individual's overall well-being. This case report focuses on
exploring a specific case of bilateral SCD.. Understanding the
implications of B-SCD on different aspects of individuals' lives,
including auditory function, balance, and overall participation, is
crucial. The International Classification of Functioning, Disability,
and Health (ICF) framework is employed, allowing for a comprehensive
evaluation of functioning status while considering contextual and
personal factors\textsuperscript{2, 3}.

Through examining vestibular outcomes, including vestibular tests and
assessments, valuable insights can be gained into the impact of B-SCD on
balance and spatial orientation. Computed tomography(CT) findings
further aid in understanding the anatomical abnormalities within the
semicircular canals, contributing to the underlying pathology of the
condition\textsuperscript{4}. The audiometric analysis is crucial in
evaluating auditory function and associated symptoms in individuals with
B-SCD. By considering audiometric test results and related measurements,
the impact this condition on hearing abilities and communication skills
can be assessed\textsuperscript{1}.

Incorporating the ICF framework into the analysis of B-SCD enables a
comprehensive evaluation of functioning implications across various
domains, offering a holistic understanding of the condition. This
approach contributes to developing tailored management strategies to
address the specific needs of individuals with B-SCD \textsuperscript{2,
3}. This case report aims to explore the vestibular outcomes, CT
findings, and audiometric analysis of individuals with B-SCD. By
utilizing the ICF framework, valuable insights into the functioning
implications of the condition can be gained. This knowledge is a
foundation for developing effective management strategies tailored to
the needs of individuals with otoneurologic diseases.

\textbf{Case presentation}

A 41-year-old male patient with no significant medical history. The
patient sought consultation due to auditory discomfort, experiencing
bilateral hyperacusis (increased sensitivity to sound), dizziness, and
vertigo when pronouncing certain phonemes, including instances when
water from the shower falls on the head.

\textbf{Methods}

A Neurotologist specialist examination revealed no abnormalities. The
auditory evaluation confirmed bilateral conductive hearing loss,
particularly affecting the airway thresholds between 125 to 1000 Hz
frequencies, with ascending curves. Also, the bone conduction study
showed thresholds below 0 dB at 250, 500, and 1000 Hz. Video Oculo
Nystagmography (VNG) was conducted, including analysis of spontaneous
nystagmus with and without ocular fixation and assessment of eye
movements such as slow tracking and saccades. The results were within
normal limits. Additionally, video head impulse test(v-Hit) testing
yielded normal findings. Cervical vestibular myogenic evoked potentials
(c-VEMPs) demonstrated a significant decrease in presentation
thresholds, even occurring at stimuli below 60 dB bilaterally, with
amplitudes well above average values.

Furthermore, spontaneous nystagmus without ocular fixation was observed
during pressure stimuli using the Valsalva Maneuver and sound
stimulation while pronouncing the phoneme ''M.'' In particular, the
production of the mentioned phoneme triggered an intense horizontal
rotational nystagmus to the left, defined as ``fremitus
nystagmus''\textsuperscript{4} . Based on the findings, a diagnosis of
bilateral superior semicircular canal dehiscence (B-SCD) is proposed. A
high-resolution ear CT scan with cuts smaller than 0.5 mm is recommended
to confirm the presence of the bone defect (\textbf{Figure 1} ).

The diagnosis of B-SCD relies on a combination of characteristic
symptoms, audiometric testing, and radiographic findings. Ward et al.
established consensus criteria that include air-bone gaps on audiometry
and visible dehiscence of the superior canal on high-resolution CT
scans. Additional vestibular symptoms may include trigged dizziness or
nystagmus in response to loud noises, as noted in the case report. While
conservative treatment is reasonable in milder cases, surgery aimed at
resurfacing or plugging the dehiscent canal can relieve
symptoms\textsuperscript{4}. The described bilateral B-SCD repair
surgery improves conductive hearing loss, though vestibular disturbances
may persist\textsuperscript{3-5}.

\textbf{Discussion}

B-SCD is characterized by the absence of bone covering the superior
semicircular canals, leading to various auditory and vestibular
symptoms. Articles shed light on this syndrome. Pereira et al. (2020)
reported a case of B-SSCD with bilateral conductive hearing loss and
subtle vestibular symptoms\textsuperscript{5-6}. Bi et al. (2017)
explored superior semicircular canal dehiscense (SSCD) syndrome in
depth, discussing its diagnosis and treatment
options\textsuperscript{7}. Mercado et al. (2016) presented a case study
of SSCD syndrome, emphasizing its clinical features\textsuperscript{8}.
Lee et al. (2016) discussed a case of bilateral SSCD with the Tullio
phenomenon, where sound-induced dizziness occurs\textsuperscript{9}, as
is the case in this report.

We developed an ICF assessment sheet to analyze this case. Using
different sources of information like anamnesis- interview, WHODAS 2.0,
videonystagmography, v-Hit, c-VEMP, and CT. We created a functioning
profile (\textbf{Figure 2} )where we selected specific categories
according to patient necessities. The areas analyzed were body
functions, structures, activity and participation, and environmental
factors.

In the first domain, body functions, the patient manifests most
impairment in hearing (b230) and vestibular function(b235), moderate and
severe, respectively. We worked with the inner ear structure(s260) for
body structure; the classification was severe, the nature is ''aberrant
dimension,'' and the location is 3, meaning ''both.''

The activity and participation were analyzed according to patient
requirements; listening (d115), conversation(d350), and washing body
parts(d5100)were categories that the patient claimed like relevant in
his life and about the qualification are severe limitation in
functioning profile. According to the interview, remunerative employment
(d850) and recreation and leisure(d920) are mild restriction. In the
environmental factors, we described his facilitators as ''products and
technology for employment and precuts and technology for culture,
recreation, and sport, e135 and e140, respectively. Nevertheless, we can
describe barriers, sound (e250), and vibration(e225), two variables
essentials to consider; the first one have a mild impairment, and the
second is severe.

The use of the International Classification of Functioning, Disability,
and Health (ICF) in the field of otoneurology can be a great help in the
evaluation and clinical reasoning for diagnosis and
treatment\textsuperscript{10-13}. As previously mentioned, the patient
expressed that auditory difficulties were the most challenging,
affecting their performance and daily activities. This is supported by
evidence suggesting that patients with SSCD often experience auditory
difficulties\textsuperscript{6-8,14-15}. Although B-SCD is atypical, its
structural characteristics are clearly described\textsuperscript{16}. As
a structural condition, does its solution necessarily require a
structural approach. This is where the functioning profile of the ICF
helped guide this clinical case. The intervention focused on performance
education in daily variables such as personal hygiene, adapting work
methods, providing education about the patient's health condition, and
avoiding triggering factors such as sound and vibration. The patient was
educated about possible surgical interventions and the importance of
monitoring the dehiscence and its symptoms. Therefore, this evaluation
method is recommended for healthcare teams to guide intervention
decisions best for patients.

\textbf{Conclusion}

Utilizing the ICF framework revealed auditory limitations to be the
predominant disability, negatively affecting daily functioning,
conversations, and work. While structural aberrancy underlies the root
pathology in B-SCD, a functioning-based management plan focused on
performance education tailored to the patient's activity priorities
proved helpful. Though surgery may be indicated, conservative management
with avoidance of sound and vibration triggers and monitoring symptom
progression using the ICF functioning profile is a reasonable initial
approach, as exemplified in this case.

\textbf{Author Credit Statement}

Vijaya Prakash Krishnan Muthaiah and Ignacio Novoa Carnejo analyzed and
interpreted investigative data. Ignacio Novoa Cornejo, Victor
Mercado-Martinez, Gustavo Ulloa-Alvarado, and Carlos Pino-Urrutia
performed a collected data, and literature review and contributed to
writing the manuscript. Vijaya Prakash Krishnan Muthaiah and
Krishnamoorthy Gunasekaran contributed to the writing and review of the
manuscript. All authors read and approved the final manuscript.

\textbf{References}

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