1.Empty Sella as a Clue to Idiopathic Intracranial Hypertension Presenting with Pulsatile Tinnitus and Progressive Hearing Loss
Jean Mun Cheah ; Prasana Nair Gengadharan ; Yuan Ye Beh ; Shireene Ratna Vethakkan
Journal of the ASEAN Federation of Endocrine Societies 2026;41(S1):88-89
Introduction:
Empty sella (ES) is characterized by herniation of the
subarachnoid space into the sella turcica, leading to
pituitary flattening. Although often considered incidental,
ES is increasingly recognized as a radiological marker of
idiopathic intracranial hypertension (IIH) which classically
presents with headache and visual loss. Importantly, IIH
may also present with otolaryngological manifestations
such as pulsatile tinnitus, hearing loss, and vertigo.
Audiovestibular symptoms have been reported in up to
88.9% of patients with primary ES, with sensorineural
hearing loss being the most frequent, while pulsatile
tinnitus occurs in approximately 58% of IIH cases.
Case:
A 51-year-old female presented with 3 months of progressively worsening headaches, rapid deterioration of
left-sided hearing, and a 20-kg weight gain over 1 year. Audiometry confirmed moderate left sensorineural hearing
loss. Examination revealed a normotensive female with a
body mass index of 35 kg/m² and diplopia on left upward
gaze without evidence of ophthalmoplegia. The rest of
the neurological examination was unremarkable. There
was no evidence of papilledema or Cushingoid features.
Repeat magnetic resonance imaging brain in 2025 showed
persistent partial ES and loss of the posterior pituitary bright
spot without an intracranial mass lesion or venous sinus
thrombosis. There was no biochemical evidence of hypopituitarism or Cushing’s syndrome. Autoimmune markers
were unremarkable. Lumbar puncture demonstrated
a mildly elevated opening pressure of 23 mmHg with
normal CSF composition. Based on the clinical and radiological findings, early IIH
was diagnosed. She was treated with acetazolamide, and
prescribed tirzepatide for weight reduction, resulting in
improvement in headache and tinnitus.
Conclusion
IIH can manifest with partial ES and audiovestibular
symptoms related to raised intracranial pressure. Early
recognition is important, and IIH should be suspected in
patients with pulsatile tinnitus or progressive sensorineural
hearing loss. Acetazolamide and weight management
remain the mainstay of treatment, with emerging evidence
supporting a potential role for glucagon-like peptide-1
receptor agonists in reducing intracranial pressure.
Pseudotumor Cerebri
;
Tinnitus
;
Hearing Loss
2.Clinical Characteristics and Outcomes of Generalized Myasthenia Gravis in Malaysia: A Single-Center Experience
Jie Ying TAN ; Cheng Yin TAN ; Prasana Nair GENGADHARAN ; Nortina SHAHRIZAILA ; Khean Jin GOH
Journal of Clinical Neurology 2024;20(4):412-421
Background:
and Purpose Myasthenia gravis (MG) is clinically heterogeneous and can be classified into subgroups according to the clinical presentation, antibody status, age at onset, and thymic abnormalities. This study aimed to determine the clinical characteristics and outcomes of generalized MG (GMG) patients based on these subgroups.
Methods:
Medical records of MG patients from 1976 to 2023 were reviewed retrospectively.Patients with pure ocular MG were excluded. Data on demographic, clinical characteristics, laboratory features, and outcomes were analyzed.
Results:
This study included 120 GMG patients. There was a slight preponderance of female patients over male patients (male:female ratio=1:1.3), with the age at onset exhibiting a bimodal distribution. Female patients peaked at a lower age (21–30 years) whereas male patients peaked at a higher age (61–70 years). Most (92%, 105 of 114) patients had positive anti-acetylcholine receptor antibodies. Five patients were also tested for anti-muscle-specific tyrosine kinase antibodies, with two showing positivity. Thymectomy was performed in 62 (52%) patients, of which 30 had thymoma, 16 had thymic hyperplasia, 7 had an involuted thymus, and 6 had a normal thymus. There were significantly more female patients (68% vs. 45%, p=0.011) with early-onset disease (<50 years old) and thymic hyperplasia (33% vs. 0%, p<0.025). Most (71%) of the patients had a good outcome based on the Myasthenia Gravis Foundation of America postintervention status. GMG patients with early-onset disease had a significantly better outcome than patients with a late onset in univariate (58% vs. 37%, p=0.041) and multivariate (odds ratio=4.68, 95% confidence interval=1.17–18.64, p=0.029) analyses.
Conclusions
Female patients with early-onset MG and thymic hyperplasia had significantly better outcomes, but only early-onset disease was independently associated with a good outcome. These findings are comparable with those of other studies.


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